We report the anesthetic management of a 7-month-old male infant with severe aortic regurgitation (AR) scheduled for the Ross procedure. To the best of our knowledge, this is the first report from the viewpoint of anesthetic management for the Ross procedure performed in an infant. He had been suffering from severe AR that occurred suddenly when he was 5 months old. The cause of the AR was considered to be spontaneous rupture of a fenestrated aortic valve, owing to congenital tissue defect in part of the aortic valvular leaflet. The Ross procedure was scheduled to be performed under general anesthesia using deep hypothermic cardiopulmonary bypass (CPB). Continuous infusion of nitroglycerin was started during CPB and continued after CPB to dilate the newly implanted coronary arteries for the prevention of myocardial ischemia and to decrease afterload and pulmonary vascular resistance. Weaning from CPB was performed without difficulty, but after the prolonged CPB he had a bleeding tendency that needed transfusion and a hemostatic drug. Monitoring with transesophageal echocardiography was very useful for evaluating myocardial ischemia, and for assessing the procedure and the completion of surgical repair. His postoperative course was uneventful and he was discharged on the 25th postoperative day.