Moyamoya disease is a rare, progressive, occlusive cerebrovascular disorder characterized by bilateral stenosis of the intracranial portion of the internal carotid artery. Symptoms including transient ischemic attacks, seizures, intracranial hemorrhage and cerebral infarction, are variable. There are few case reports of moyamoya disease in pregnancy. Since hyperventilation-induced cerebral ischemia and hypertension are provoked by active labor, cesarean section has been recommended to avoid neurologic complications for pregnant women with moyamoya disease. The optimal anesthetic management for cesarean section in these patients has not been discussed. We report a case of moyamoya disease in a patient presenting for cesarean section at 40 weeks' gestation. Epidural anesthesia was administered using 0.5% bupivacaine and fentanyl. Intraoperative hemodynamic state was stable. The patient has no significant postoperative complications.