Based on a study of 20 patients operated on between the ages of 21 and 38 for congenital valvular aortic stenosis, the distinguishing features of congenital aortic valvular stenosis in adults are reviewed: valvular calcification (75%), valvular dome rarely present (10%), usually moderate cardiac disability (70%), diminished or inaudible second heart sound (50%), associated diastolic murmur (75%), electrocardiographic left ventricular hypertrophy (70%), and an infrequent protosystolic click (30%). Surgery is necessary for symptomatic patients. If patients are asymptomatic, surgery is decided after measuring the left ventricle-aortic pressure gradient by continuous Doppler wave study, or by cardiac catheterization. If surgery is not performed, a regular follow-up is necessary because stenosis can evolve. Valvular replacement by a mechanical valve is preferable. Even if the aortic valve is not calcified and a simple commissurotomy is technically possible, it is only a palliative method with unsatisfactory results and an annual mortality rate after surgery of 1%.