Moyamoya disease is characterized by a narrowing or occlusion of both internal carotid arteries in the presence of an abnormal hemangiomatosis vascular network at the base of the brain. These patients increasingly present for surgical management to improve the cerebral circulation. However, surgery may be complicated by cerebral ischemia, and thus patients require particular care during the perioperative period. The risk factors of perioperative ischemic complications include the presence of a preoperative low density area by computed tomography, the occurrence of frequent preoperative transient ischemic attacks, or hypocapnia or hypotension. We describe one case of general anesthesia for superficial temporal artery to middle cerebral artery anastomosis (STA-MCA) with encephalomyosynangiosis (EMS) in a moyamoya disease patient. The patient expired because of a developing cerebral infarction on the fifth postoperative day.