Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries, but the etiology remains unclear. Angiographic characteristics include bilateral stenosis or occlusion of the terminal portions of the intracranial internal carotid arteries and bilateral development of fine collateral vessels at the base of the brain known as ‘Moyamoya vessels’. Cardiac surgery using cardiopulmonary bypass due to coronary artery disease and others among patients with moyamoya disease is very rare, and cardiac surgery for such patients has a potential risk of intraoperative and perioperative brain ischemia. We successfully treated a patient who underwent artrial septal defect closure and coronary artery bypass graft using the cardiopulmonary bypass, so we report this case with a brief literature review.