To observe the therapeutic effect and safety of rituximab (anti-CD20 monoclonal antibody) in the treatment of refractory autoimmune hemolytic anemia (AIHA). One AIHA patient refractory to corticosteroid and splenectomy was treated with rituximab, 375 mg/m(2) weekly for four times. Her hemolytic symptoms, adverse effects, hemoglobin (Hb) concentration and other laboratory data were monitored. The results showed that concentration of lactic dehydrogenase (LDH), total bilirubin (TBIL) and indirect bilirubin (IBIL) began to decrease at 11 days after the first dose of rituximab, and decreased to normal range after 45 days. Concentration of hemoglobin increased up to 95 - 100 g/L. The patient remained disease-free 4 months after treatment. No adverse effect was found during the process of treatment. It is concluded that anti-CD20 monoclonal antibody (rituximab) is both effective and safe for the treatment of refractory autoimmune hemolytic anemia.