Aortopulmonary septal defect, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the aorta comprised a challenging constellation of defects in a 13-day-old neonate, who underwent complete repair without homograft tissue or synthetic graft. After the aorta was separated from the pulmonary artery, the descending aorta was anastomosed to the septal defect in the ascending aorta. The right pulmonary artery was anastomosed to the septal defect in the main pulmonary artery, anterior to the aorta. The postoperative course was uncomplicated, and the potential for growth of both great vessels was optimized.