Neuroblastoma is a cancer of the sympathetic nervous system, typically diagnosed in very young children with a median age of 17 months. It can present as a mass in various parts of the body and has a highly variable clinical course, ranging from asymptomatic to critical illness due to local invasion or disseminated disease. Despite its high mortality, neuroblastoma has a high rate of spontaneous regression, and survival rates have improved over the years. Genetic factors play a significant role in the development and prognosis of neuroblastoma, with mutations in the *ALK* and *PHOX2B* genes being the most common. The International Neuroblastoma Risk Group (INRG) classification system uses 13 prognostic factors to categorize patients into very low, low, intermediate, and high risk, with corresponding 5-year event-free survival rates of >85%, >75 to ≤85%, ≥50 to ≤75%, and <50%, respectively. Treatment strategies are tailored to these risk categories, with high-risk cases requiring more intensive therapy. Recent advances include the use of targeted therapies, such as ALK inhibition, and immunotherapeutic approaches, such as anti-GD2 monoclonal antibodies. The future holds promise for more precise and effective treatments, driven by a deeper understanding of the molecular mechanisms underlying neuroblastoma.Neuroblastoma is a cancer of the sympathetic nervous system, typically diagnosed in very young children with a median age of 17 months. It can present as a mass in various parts of the body and has a highly variable clinical course, ranging from asymptomatic to critical illness due to local invasion or disseminated disease. Despite its high mortality, neuroblastoma has a high rate of spontaneous regression, and survival rates have improved over the years. Genetic factors play a significant role in the development and prognosis of neuroblastoma, with mutations in the *ALK* and *PHOX2B* genes being the most common. The International Neuroblastoma Risk Group (INRG) classification system uses 13 prognostic factors to categorize patients into very low, low, intermediate, and high risk, with corresponding 5-year event-free survival rates of >85%, >75 to ≤85%, ≥50 to ≤75%, and <50%, respectively. Treatment strategies are tailored to these risk categories, with high-risk cases requiring more intensive therapy. Recent advances include the use of targeted therapies, such as ALK inhibition, and immunotherapeutic approaches, such as anti-GD2 monoclonal antibodies. The future holds promise for more precise and effective treatments, driven by a deeper understanding of the molecular mechanisms underlying neuroblastoma.