BackgroundDespite several previous reports, there are no established procedures for intraoperative management in moyamoya disease patients requiring cardiac surgery.Case presentationHerein, we report the case of a 42-year-old man who was scheduled to undergo mitral valve plasty for severe mitral regurgitation. He had been diagnosed with moyamoya disease on the onset of cerebral ischemia at 41 years of age. During the cardiac surgical procedure, the patient was maintained on inhalation anesthesia with 1 to 1.5 % sevoflurane. Sevoflurane causes cerebral vasodilation followed by increased cerebral blood flow, and moreover we expected a sevoflurane preconditioning-induced neuroprotective effect. In addition, we used pulsatile perfusion support to maintain cerebral circulation with intra-aortic balloon pumping during the cardiopulmonary bypass. We aimed to keep the mean arterial pressure constantly above 70 mmHg. We were able to maintain regional cerebral oxygen saturation at 80 % of the baseline value, and could not detect the progression of neurological deficits using follow-up brain single photon emission computed tomography. The patient was discharged 16 days after admission.ConclusionsThe details of the clinical course of his case will add to our knowledge regarding intraoperative management options in moyamoya disease patients requiring cardiac surgery. We suggest that pulsatile blood flow supported by intra-aortic balloon pumping and sevoflurane anesthesia for increasing cerebral blood flow and for possible neuroprotection may be efficacious for anesthetic management of moyamoya disease patients.