Moyamoya disease is a cerebrovascular condition, characterized by chronic progressive stenosis of the terminal portion of the bilateral internal carotid arteries or the proximal portion of the anterior and/or middle cerebral arteries, which leads to the formation of an abnormal vascular network composed of collateral pathways at the base of the brain. Moya-Moya disease has been associated with multiple immune disorders, however, only one case of acquired thrombotic thrombocytopenic purpura has been documented in association with this disease, and none associated with antiphospholipid syndrome. We are presenting a challenging case where all these three medical conditions were present at the same time.