Four to seven percent of infants born with aortic atresia have a normal-sized left ventricle in association with a ventricular septal defect and a normal mitral valve. In contrast to the more common group of infants with aortic atresia whose left ventricle is hypoplastic, this important subgroup has potential for complete operative correction involving both the right and left ventricle. Our approach was to perform complete repair at one stage. The patient who underwent single-stage repair was discharged from the hospital in good condition. Achieving a physiologically normal circulation simplifies the postoperative management of this condition.