Only three cases of the combination of bicuspid aortic valve and ruptured aneurysm of the sinus of Valsalva, associated with previously repaired coarctation of aorta, have been reported. A twenty-year-old man with a sudden onset of CHF due to ruptured aneurysm of the sinus of Valsalva underwent intracardiac repair by direct closure of the sinus Valsalva in combination with patch closure of a subarterial VSD. Although, no AR was detected preoperatively, massive reguratitation occurred after the repair due to subsequent failure of aortic valve coaptation in the present of the bicuspid aortic valve, which was not diagnosed preoperatively. Aortic valve replacement with SJM 25 mm was successfully performed.