We report a patient with longstanding systemic lupus erythematosus (SLE) who developed pure red cell aplasia (PRCA). This condition is rare in connective tissue diseases and is reported in 32 previous cases of SLE in literature. Our patient recovered, apparently in response to treatment with high dosage of corticosteroids, but relapse occurred when the prednisone dosage was tapered down to 10 mg/day. The patient was successfully treated with cyclosporin A with no recurrence of the disease in the last 2 years.