Thrombotic thrombocytopenic purpura is an uncommon disease and is rare in systemic lupus erythematosus. Rarely, patients do not respond to plasma exchange therapy, and treatment options are limited and often disappointing, thus it is a therapeutic challenge to clinicians. We report a patient with lupus nephritis who developed refractory thrombotic thrombocytopenic purpura and was subsequently treated with cyclosporin A. An immediate and sustained response was demonstrated, and she remained well; eventually she was able to be cyclosporin free after the acute episode. Cyclosporin A can be a safe and effective therapeutic option in patients with refractory thrombotic thrombocytopenic purpura.