Although rare, visceroatrial situs inversus is not exceptional in patients with complex congenital heart defects. Achieving orthotopic heart transplantation using a graft harvested in a donor with situs solitus is surgically demanding. Technical problems can, however, be overcome by adequate harvesting of donor heart and by the use of innovative reimplantation techniques. These include: separate right and left pulmonary venous anastomoses, creation of an atrio-pericardial tunnel for reimplantation of the inferior vena cava, extracardiac reconstruction of the superior vena cava pathway using the donor innominate vein, direct aortic and pulmonary arterial reconstruction after adequate mobilization. There is evidence that the early risk of heart transplantation is not increased by the presence of recipient situs inversus.