Moyamoya disease is a rare affliction, which progressively affects cerebral arteries, more frequently those in the circle of Willis and the internal carotid arteries. This condition has a genetic component, but the cause of the disease is unknown. It usually affects children but can affect adults as well, representing a health issue due to its mortality. Not in a few cases, Moyamoya disease has been difficult to recognize as a cause of ischemic or hemorrhagic strokes. In this article, we present the case of a young patient, with a medical history of cerebral hemorrhage, which was diagnosed with Moyamoya disease after clinical and cerebral angiographic corroboration in a specialized vascular center. The patient entered the Emergency Receiving Unit with a sudden comatose state. Paraclinical tests revealed massive intraparenchymal hemorrhage, with movement in the medial line and ventricular flooding.