Acute pancreatitis can be encountered as a rare complication of thrombotic thrombocytopenic purpura and it has been associated with recurrent thrombotic thrombocytopenic purpura. In this report, we present a case of thrombotic thrombocytopenic purpura closely followed for recurrent acute pancreatitis attacks and having a lethal clinical picture due to splenic vein rupture in spite of plasmapheresis and steroid treatments.