Despite incomplete understanding of the pathology of Hodgkin's disease, its cell of origin, and the molecular events that induce its malignant transformation, most children diagnosed with Hodgkin's disease will be long-term survivors. Many controversies still exist regarding the optimal staging and treatment of children, which has evolved over the past 30 years owing to the pioneering efforts of pediatric investigators to reduce late treatment sequelae. Today, most institutional protocols prescribe multiagent chemotherapy, either alone or in conjunction with low-dose involved-field radiation therapy for children in whom growth and development is still an issue. Recent studies indicate that “risk-adapted” combined modality regimens of involved-field radiotherapy and three to four cycles of chemotherapy produce cure rates comparable with those using similar radiotherapy and six cycles, with fewer acute and late treatment sequelae in children with localized disease. Identification of patients at risk for treatment failure who may benefit from intensification of therapy remains a challenge for the future. Similarly, better understanding of the pathogenesis of Hodgkin's disease may provide the basis for novel therapies that improve cure rates and reduce treatment toxicity.