Clinical thrombotic thrombocytopenic purpura (TTP) is characterized by a pentad of microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, renal involvement, and fever. A case of TTP in which early symptoms and signs were suggestive of ischemic heart disease, renal failure, and severe thrombocytopenia developed to a rapid outcome of death. The postmortem examination revealed coronary artery microthrombi, typical of TTP. The clinical presentation of this TTP was atypical: severe thrombocytopenia, striking renal and CNS symptoms were present, but fever and anemia were not present. Thrombotic thrombocytopenic purpura is an uncommon condition that carries a high fatality rate if untreated. Awareness of this syndrome and its high risk of sudden death underlines the importance of rapid diagnosis and treatment.