Thrombotic thrombocytopenic purpura (TTP) is still a life threatening disease, despite vast developments in treatment modalities. We report a case of severe TTP who did not respond to high dose intravenous steroids, plasmapheresis, defibrotide and splenectomy. However, after splenectomy with plasma replacement and intravenous immune globin infusion as a maintenance therapy, she improved and had durable complete remission. This observation led us to consider the importance of maintenance treatment in frequently relapsing TTP cases.