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Thrombotic thrombocytopenic purpura (TTP) is a life-threatening multisystem disorder of unknown etiology, first described by Moschkowitz in 1924. TTP is a thrombotic microangiopathy characterized by microvascular lesions with platelet aggregation. TTP is more common in adults and is associated with pregnancy; diseases such as HIV, cancer, bacterial infection, and vasculitis; bone marrow transplantation; and drugs TTP is a hematologic emergency. It is a multisystem disease that can cause rapid deterioration of the patient's neurologic, renal, and hematologic status. TTP is an uncommon disease with a high fatality rate if untreated or misdiagnosed. Rapid diagnosis and aggressive treatment by therapeutic plasma exchange are necessary to reduce the risk of a fatal outcome. Current clinical criteria for initiating therapy are: thrombocytopenia, and absence of other disease entities that could explain the thrombocytopenia. Early recognition and management are essential for patient survival. TTP is difficult to diagnose because the patient's presentation can be nonspecific and the characteristic pentad of symptoms may not occur together. Other disease entities can have some of the same symptoms. We discuss a case report of Moschkowitz syndrome in Emergency Department.