Anatomically corrected malposition of the great arteries (ACMGA) is rare congenital cardiac anomaly. In a heart with ACMGA, both great arteries arise from the appropriate ventricle but they exit parallel to each other. The pulmonary trunk is located posterior and to the right of the aorta. ACMGA associated with tubular hypoplasia of the aortic arch is an extremely rare cardiac malformation and it has previously been reported in only four patients. In addition, successful surgical repair for this combination of anomalies was achieved in only one patient. We describe clinical and morphologic features in a neonate with this complex anomaly. The patient successfully underwent the staged operation.