A 13-week-old baby was referred with dextrocardia, situs inversus, transposition of the great arteries, intact ventricular septum, patent foramen ovale, right aortic arch with severe preductal aortic coarctation and large patent ductus arteriosus. Left ventricular mass index as well as thickness was adequate, 118 g/m2 and 5.9 mm, respectively; thus, a primary arterial switch with aortic coarctation repair was performed. The patient made a full recovery without the need for extracorporeal life support. Adequate left ventricular mass index and thickness in late-presenting transposition of the great arteries with intact ventricular septum might justify primary arterial switch.