Interruption of the aortic arch, distal aortopulmonary septal defect, and aortic origin of the right pulmonary artery with intact interventricular septum were associated findings in a 4-month-old infant who underwent successful one-stage repair without the use of any synthetic material, thus allowing for a potential for growth of both the pulmonary artery and the aorta. The baby made an uneventful recovery and remains asymptomatic 36 months after repair.