Aortic atresia is a form of congenital cardiac disease complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. Occasionally, neonates with severe aortic stenosis have associated severe hypoplasia of the ascending aorta and left ventricle. These two defects constitute the most prevalent forms of so-called hypoplastic left heart syndrome. At present, this lesion is universally fatal with no established surgical management. This report describes our experience with the evolution and evaluation of staged surgical management of infants with hypoplastic left heart syndrome.