Transposition of the great arteries with intact ventricular septum and aortopulmonary window is an extremely rare anatomic combination, having been reported just twice previously. Other authors performed a physiologic repair, because the combination was considered unsuitable for an anatomic repair. We describe the case of a 26-day-old baby with such anatomy who was successfully treated with an arterial switch operation. A 4 mm fenestration at atrial level was made for a smoother postoperative course.