We report a 16-day-old boy who had transposition of great arteries combined with interrupted aortic arch (IAA). The boy developed cyanotic lips and body bruising 2 h after birth. Color Doppler sonography showed transposition of great arteries. The patient underwent total surgical correction of the transfection and defects. We summarized our experience in the perioperative management of the patient, including maintenance of body temperature, close intraoperative monitoring, anesthesia management, cardiopulmonary bypass (CPB) management and so on.