Cystic echinococcosis (CE), caused by the larval stage of Echinococcus granulosus, is a zoonotic disease affecting both humans and animals, with significant public health implications. Despite being classified as a neglected tropical disease, CE remains a major global health concern due to its zonal endemicity and potential for severe morbidity. The World Health Organization (WHO) has identified CE as a priority for control, emphasizing the need for integrated One Health approaches to address the disease's impact on humans, animals, and the food chain. However, data on CE are limited, and research on its geographical distribution is ongoing. This review summarizes recent findings on CE, including its taxonomy, epidemiology, and management strategies, and correlates these with WHO recommendations to highlight current efforts and areas needing improvement. The study analyzed 751 research articles, narrowing them down to 52 that met inclusion criteria. Key findings include the classification of CE cysts into five types and three groups, with CE1 and CE2 representing active infections, CE3 as a transitional stage, and CE4 and CE5 as inactive. CE is diagnosed using imaging, serology, and clinical presentation, with ultrasonography being the gold standard for abdominal CE. Serological tests such as ELISA and immunochromatography are used in conjunction with imaging for diagnosis. The genus Echinococcus has undergone taxonomic revisions, with E. granulosus sensu lato (s.l.) encompassing multiple genotypes, including E. granulosus sensu stricto (s.s.). Epidemiologically, CE is endemic in Europe, Africa, Central Asia, the Middle East, and parts of South America and Australia, with varying prevalence rates. The WHO estimates an incidence of over 50 per 100,000 person-years and a prevalence of 5–10% in some regions. Recent studies show a decline in prevalence in certain areas, such as Turkana, Kenya. The disease is transmitted through the fecal-oral route, with environmental contamination playing a significant role. Prevention and control strategies include vaccination of sheep, deworming dogs, and public health programs. Treatment options include surgery, chemotherapy, and minimally invasive procedures like PAIR. The WHO recommends a multidisciplinary approach for managing CE, emphasizing the need for improved surveillance, diagnostic tools, and control measures. Notable projects, such as the HERACLES and mEmE initiatives, have contributed to CE research, improving surveillance, diagnostic methods, and understanding of the disease's epidemiology. These projects align with the WHO's road map for neglected tropical diseases, highlighting the importance of international collaboration and standardized protocols. Despite these efforts, challenges remain in data collection, underreporting, and the need for more effective interventions. The review underscores the importance of continued research, improved diagnostics, and integrated One Health strategies to effectively control and prevent CECystic echinococcosis (CE), caused by the larval stage of Echinococcus granulosus, is a zoonotic disease affecting both humans and animals, with significant public health implications. Despite being classified as a neglected tropical disease, CE remains a major global health concern due to its zonal endemicity and potential for severe morbidity. The World Health Organization (WHO) has identified CE as a priority for control, emphasizing the need for integrated One Health approaches to address the disease's impact on humans, animals, and the food chain. However, data on CE are limited, and research on its geographical distribution is ongoing. This review summarizes recent findings on CE, including its taxonomy, epidemiology, and management strategies, and correlates these with WHO recommendations to highlight current efforts and areas needing improvement. The study analyzed 751 research articles, narrowing them down to 52 that met inclusion criteria. Key findings include the classification of CE cysts into five types and three groups, with CE1 and CE2 representing active infections, CE3 as a transitional stage, and CE4 and CE5 as inactive. CE is diagnosed using imaging, serology, and clinical presentation, with ultrasonography being the gold standard for abdominal CE. Serological tests such as ELISA and immunochromatography are used in conjunction with imaging for diagnosis. The genus Echinococcus has undergone taxonomic revisions, with E. granulosus sensu lato (s.l.) encompassing multiple genotypes, including E. granulosus sensu stricto (s.s.). Epidemiologically, CE is endemic in Europe, Africa, Central Asia, the Middle East, and parts of South America and Australia, with varying prevalence rates. The WHO estimates an incidence of over 50 per 100,000 person-years and a prevalence of 5–10% in some regions. Recent studies show a decline in prevalence in certain areas, such as Turkana, Kenya. The disease is transmitted through the fecal-oral route, with environmental contamination playing a significant role. Prevention and control strategies include vaccination of sheep, deworming dogs, and public health programs. Treatment options include surgery, chemotherapy, and minimally invasive procedures like PAIR. The WHO recommends a multidisciplinary approach for managing CE, emphasizing the need for improved surveillance, diagnostic tools, and control measures. Notable projects, such as the HERACLES and mEmE initiatives, have contributed to CE research, improving surveillance, diagnostic methods, and understanding of the disease's epidemiology. These projects align with the WHO's road map for neglected tropical diseases, highlighting the importance of international collaboration and standardized protocols. Despite these efforts, challenges remain in data collection, underreporting, and the need for more effective interventions. The review underscores the importance of continued research, improved diagnostics, and integrated One Health strategies to effectively control and prevent CE