Endometriosis, a common benign condition affecting reproductive-aged women, is associated with an increased risk of developing endometriosis-associated ovarian cancers (EAOCs), including endometrioid carcinomas (EnOCs) and clear cell ovarian carcinomas (CCCs). Atypical endometriosis (AE), characterized by cytological and architectural atypia, is considered a precursor to EAOC. This narrative review aims to provide an overview of EAOC, including its pathogenesis, diagnostic process, and therapeutic options, proposing a pragmatic clinical flowchart to guide decision-making. The review includes 81 articles, highlighting the importance of ultrasound imaging in differentiating typical endometriosis from AE and EAOC. The presence of AE is a significant risk factor for EAOC, with approximately 60-80% of EAOC cases occurring with AE. Molecular alterations, such as *ARID1A*, *PIK3CA*, and *PTEN* mutations, are common in EAOC and may serve as biomarkers for early detection. However, the clinical application of these biomarkers is challenging due to the need for molecular analysis. The review also discusses the role of surgery in managing EAOC, emphasizing the importance of identifying high-risk lesions to prevent intraoperative dissemination of malignant cells. Conservative pharmacological approaches are generally preferred, but surgical intervention may be necessary in certain cases. The decision to perform surgery should consider factors such as age, medical history, and patient desires. Despite the increased risk of EAOC in endometriosis patients, early detection and risk stratification remain challenging. Further research is needed to improve understanding of the pathogenesis and to develop more effective diagnostic and therapeutic strategies.Endometriosis, a common benign condition affecting reproductive-aged women, is associated with an increased risk of developing endometriosis-associated ovarian cancers (EAOCs), including endometrioid carcinomas (EnOCs) and clear cell ovarian carcinomas (CCCs). Atypical endometriosis (AE), characterized by cytological and architectural atypia, is considered a precursor to EAOC. This narrative review aims to provide an overview of EAOC, including its pathogenesis, diagnostic process, and therapeutic options, proposing a pragmatic clinical flowchart to guide decision-making. The review includes 81 articles, highlighting the importance of ultrasound imaging in differentiating typical endometriosis from AE and EAOC. The presence of AE is a significant risk factor for EAOC, with approximately 60-80% of EAOC cases occurring with AE. Molecular alterations, such as *ARID1A*, *PIK3CA*, and *PTEN* mutations, are common in EAOC and may serve as biomarkers for early detection. However, the clinical application of these biomarkers is challenging due to the need for molecular analysis. The review also discusses the role of surgery in managing EAOC, emphasizing the importance of identifying high-risk lesions to prevent intraoperative dissemination of malignant cells. Conservative pharmacological approaches are generally preferred, but surgical intervention may be necessary in certain cases. The decision to perform surgery should consider factors such as age, medical history, and patient desires. Despite the increased risk of EAOC in endometriosis patients, early detection and risk stratification remain challenging. Further research is needed to improve understanding of the pathogenesis and to develop more effective diagnostic and therapeutic strategies.