Neuroblastoma is an embryonal tumor of the autonomic nervous system, typically occurring in young children with a median age of 17 months. It arises in the sympathetic nervous system, commonly in the adrenal medulla or paraspinal ganglia, and can present as a mass in the neck, chest, abdomen, or pelvis. The disease has variable clinical presentations, ranging from asymptomatic masses to life-threatening conditions due to local invasion or metastasis. Neuroblastoma is the most common cancer in infants under one year of age, with an incidence of 10.2 cases per million children under 15 years. Over the past century, neuroblastoma has shown diverse clinical behaviors, with outcomes improving significantly, particularly for less aggressive cases. However, high-risk neuroblastoma remains challenging, with only modest improvements in survival despite intensified therapies. Genetic factors play a crucial role, with mutations in the ALK and PHOX2B genes being associated with familial cases. Sporadic cases are influenced by common DNA variations, and genome-wide association studies have identified genetic markers linked to neuroblastoma susceptibility. Risk stratification is based on clinical and biological factors, including age, tumor stage, and genetic features. The International Neuroblastoma Risk Group (INRG) classification system divides patients into 16 risk groups based on 13 prognostic factors. High-risk cases are characterized by aggressive tumor biology, such as MYCN amplification, which is a key biomarker for treatment decisions. Treatment for high-risk neuroblastoma involves three phases: induction of remission, consolidation, and maintenance. Chemotherapy regimens, including cisplatin, etoposide, and topotecan, are used to induce remission. Consolidation with myeloablative therapy and autologous stem cell rescue has shown promise. Immunotherapy, such as anti-GD2 monoclonal antibodies, has been explored, with some studies showing improved survival when combined with cytokines. Despite advances, relapse remains common, and new therapies are being developed to improve outcomes. Targeted therapies, such as ALK inhibitors, are being tested in clinical trials. Research into genetic and molecular markers continues to refine risk stratification and treatment strategies. Ongoing studies aim to identify more precise biomarkers and develop safer, more effective treatments for neuroblastoma.Neuroblastoma is an embryonal tumor of the autonomic nervous system, typically occurring in young children with a median age of 17 months. It arises in the sympathetic nervous system, commonly in the adrenal medulla or paraspinal ganglia, and can present as a mass in the neck, chest, abdomen, or pelvis. The disease has variable clinical presentations, ranging from asymptomatic masses to life-threatening conditions due to local invasion or metastasis. Neuroblastoma is the most common cancer in infants under one year of age, with an incidence of 10.2 cases per million children under 15 years. Over the past century, neuroblastoma has shown diverse clinical behaviors, with outcomes improving significantly, particularly for less aggressive cases. However, high-risk neuroblastoma remains challenging, with only modest improvements in survival despite intensified therapies. Genetic factors play a crucial role, with mutations in the ALK and PHOX2B genes being associated with familial cases. Sporadic cases are influenced by common DNA variations, and genome-wide association studies have identified genetic markers linked to neuroblastoma susceptibility. Risk stratification is based on clinical and biological factors, including age, tumor stage, and genetic features. The International Neuroblastoma Risk Group (INRG) classification system divides patients into 16 risk groups based on 13 prognostic factors. High-risk cases are characterized by aggressive tumor biology, such as MYCN amplification, which is a key biomarker for treatment decisions. Treatment for high-risk neuroblastoma involves three phases: induction of remission, consolidation, and maintenance. Chemotherapy regimens, including cisplatin, etoposide, and topotecan, are used to induce remission. Consolidation with myeloablative therapy and autologous stem cell rescue has shown promise. Immunotherapy, such as anti-GD2 monoclonal antibodies, has been explored, with some studies showing improved survival when combined with cytokines. Despite advances, relapse remains common, and new therapies are being developed to improve outcomes. Targeted therapies, such as ALK inhibitors, are being tested in clinical trials. Research into genetic and molecular markers continues to refine risk stratification and treatment strategies. Ongoing studies aim to identify more precise biomarkers and develop safer, more effective treatments for neuroblastoma.