Pure red cell aplasia (PRCA) is an unusual early manifestation of systemic lupus erythematosus (SLE). Review of the 13 cases in literature revealed that the immune system played a significant role in disease pathogenesis. Immunosuppression has been thought to be the cause of the efficacy of corticosteroids in treatment. The case of a 28-year-old woman with SLE is described. She presented with PRCA, but has had good response to corticosteroid therapy.