Haematological involvement occurs frequently in childhood onset systemic lupus erythematosus (SLE) with autoimmune haemolytic anaemia occurring in 10%. Conventional treatment includes high-dose steroids. Second line agents used in refractory cases include azathioprine, mycophenolate mofetil, cyclosporine and cyclophosphamide. Rituximab, anti-CD20 monoclonal antibody, is emerging in the treatment of SLE refractory to conventional therapy. We report the successful use of anti-CD20 therapy in the treatment of acute life threatening haemolytic anaemia, complicating childhood onset SLE. We propose that earlier introduction of rituximab may reduce the side effects related to other cytotoxic therapy mainly steroids and cyclophosphamide. However, in view of controversies in the reported side effects of rituximab in childhood onset SLE, multicentred randomised controlled trials are required to determine the long-term efficacy, safety and optimal dosing of rituximab in childhood onset SLE.