Thrombotic thrombocytopenic purpura (TTP) is a rare disorder with an estimated incidence of 3 - 7/1,000,000. It is an autoimmune disorder characterized by fever, neurological signs, microangiopathic hemolytic anemia, thrombocytopenia and renal failure. This case report will describe a young lady who presented with acute middle cerebral artery infarct and was subsequently diagnosed to have TTP. Therapeutic plasma exchange (TPE) did not improve the neurological deficit. This case highlights the importance of recognizing TTP as a possible differential diagnosis in young onset stroke.