Moyamoya disease (MMD) is a progressive cerebrovascular occlusive disease of the internal carotid arteries and anterior and middle cerebral arteries. Non-neurological surgery in patients with MMD is often complicated by cerebral ischemia or infarction. The goals of perioperative management are to maintain normotension, normocarpnia, normovolemia and normothermia. Here we report a case of a patient with MMD who underwent patch closure of an atrial septal defect and pulmonary valvotomy by use of a normothermic and non-hemodiluted cardiopulmonary bypass. To prevent intraoperative neurological complications we performed total intravenous anesthesia with propofol, made burst suppression in EEG and monitored the jugular bulb oxygen saturation (SjvO₂) for cerebral desaturation.