A case of distal aortopulmonary septal defect associated with aortic origin of right pulmonary ar- tery, patent ductus arteriosus and hypoplasia of aortic isthmus in a 50-day-old female infant is presented. Ligation of patent ductus arteriosus, resection and end-to-end anastomosis of hypoplasia of aortic isthmus, implantation of rlght pulmonary artery to main pulmonary artery and autologous peri- cardial patch repair of aortopulmonary septal defect were performed under cardiopulmonary bypass as one-stage approach. Deep hypothermic total circulatory arrest was applied during repair of hypoplasia of aortic isthmus. The p stoperative course was uneventful.