Aortic valve atresia is usually associated with hypoplasia of the left ventricle and mitral valve. We report a patient presenting aortic atresia with ventricular septal defect and a normally sized left ventricle. The electrocardiogram showed left axis deviation and left ventricular hypertrophy. The ventricular septal defect probably favored a normal growth of the left ventricle, allowing it to unload with systole.
