Three patients with double-outlet right ventricle (DORV) and left ventricular outflow tract obstruction (LVOTO) were described. In each patient, LVOTO was produced by restrictive VSD which resulted from malalignment of the conal musculature and a discrete subaortic membrane. All patients underwent surgical repair with enlargement of the ventricular septal defect and rerouting of the outflow tract from the left ventricle to the aorta. One patient died of congestive heart failure three months postoperatively. Causes of LVOTO and surgical management of DORV with LVOTO were discussed.