Neuroblastoma is the third most common malignancy in childhood and accounts for at least 15 percent of cancer-related deaths in children. The varied clinical behavior of this disease, ranging from spontaneous regression in some to progression while under aggressive therapy in others, has been associated with various biologic differences, providing insight into pathophysiology and possibilities for more specific treatments. This article reviews the epidemiology, pathophysiology, clinical evaluation, and treatment of neuroblastoma.