Systemic lupus erythematosus (SLE) begins in childhood or adolescence in approximately 15% of cases, and although short- and long-term outcomes have improved considerably in the past 20 years there remain many areas of controversy regarding optimal management of these patients. In this chapter, areas of debate reviewed include: the appropriate timing of adding immunosuppressive therapy to corticosteroids, the optimal choice of immunosuppressive medication, the management of patients who fail 'standard' therapies, the significance and appropriate treatment of antiphospholipid antibodies in children with SLE and the emerging morbidities of childhood SLE. The goal of this chapter is to provide a balanced literature review and clinical approach to children and adolescents with SLE and to highlight areas requiring studies in paediatric clinical research.