Congenital aortic stenosis is a common pathology in adults. The valvular lesion, usually secondary to a bicuspid valve, is dominant. Dilatation of the ascending aorta, the result of a jet lesion or structural wall abnormalities, is often observed in association with the valvular stenosis. Subvalvular stenosis is progressive and may only present late, after surgery of another congenital lesion. Supravalvular stenosis is much less common and is usually diagnosed in a dysgenetic context. Echocardiography is usually diagnostic and enables quantification of the stenosis and evaluation of secondary left ventricular changes. Exercise stress testing is decisional in asymptomatic severe stenosis. Percutaneous valvuloplasty is a good palliative procedure. Other surgical techniques comprise valvular commissurotomy, supravalvular valvuloplasty, valvular replacement (autograft, homo- or heterograft or mechanical prostheses). The indications depend on the quantification of the stenosis, symptoms, the results of exercise testing, the valvular lesion secondary to subaortic stenosis and the progression of the aneurysm of the ascending aorta.