Supravalvular aortic stenosis was relatively uncommon form of congenital heart disease. This patient had typical "elfin faces" with mental retardation, and supravalvular aortic stenosis. The diagnosis was confirmed by pressure tracing obtained at retrograde left heart catheterization and aortography. The type of supravalvular aortic stenosis was localized hourglass narrowing, which was treated by insertion of prosthetic gusset placed across the area of narrowing under the cardiopulmonary bypass.ry bypass.