Thrombotic thrombocytopenia purpura (TTP) is a rare clinical syndrome characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neurologic symptoms, acute renal impairment, and fever. It has been seldom reported in systemic sclerosis (SSc). Systemic renal crisis is an infrequent complication of SSc, and is characterized by new onset malignant hypertension, rapidly progressive oliguric renal failure, and MAHA. In this study, we present a case of SSc of 1 month duration, with TTP accompanied by new onset malignant hypertension. The patient responded to plasmapheresis but still died of septic shock.