Children and adolescents represent 15-20% of all systemic lupus erythematosus (SLE) patients. Although the clinical presentation and immunological findings are similar to those of adult SLE, children usually have a more severe disease at onset with higher rates of organ involvement. Rapid diagnosis and subsequent therapy are necessary to prevent major organ damage. The survival of children with SLE has improved dramatically over the past decades due to the introduction of steroids and immunosuppressive drugs. New strategies to improve the long-term course of the disease and to reduce potential drug toxicities are necessary. A common concept does not exist. There are some promising new drugs. This review article summarizes the epidemiology, pathogenesis, clinical manifestations and therapy of childhood and adolescent-onset SLE.