A very rare case of a newborn with dextrocardia, complete transposition of the great arteries {S,D,D}, ventricular septal defect, aortic arch hypoplasia, juxtaposition of the morphologically right atrial appendage and superoinferior ventricles is herein presented. He successfully underwent arterial switch operation, ventricular septal defect closure and aortic arch reconstruction with end to end extended direct anastomosis.