Non-Hodgkin lymphoma (NHL) accounts for 7% of cancer in children and adolescents in the United States, or approximately 1000 cases annually. NHL in the pediatric population differs from that observed in adult patients with respect to staging systems, histologic subtypes of disease, treatment, and outcomes. Although more than 90% of pediatric NHL is of high-grade histology, more than 80% of patients achieve long-term event-free survival with modern therapy. This review focuses on current treatments for pediatric NHL and some of the differences between NHL observed in pediatric and adult patients.