In this paper a rare hourglass type of supravalvular aortic stenosis with normal facies and intelligence is presented. The patient was operated on utilizing a cardiopulmonary bypass and resection of the obstruction. Patch-plasty at the narrowed site of the aorta was successfully performed. This procedure relieves the high pressure on the coronary arteries and improves the left ventricular function. Therefore, the operation should be advised as soon as possible after the diagnosis is made.

Keywords: cardiac failure, congenital aortic stenosis, elfin-face syndrome, hypercalcemia, Williams syndrome