Aortic stenosis occurs in almost 5% of patients with congenital heart disease. Valvular aortic stenosis results from narrowing of the orifice size due to aberrant valve leaflets and is frequently associated with other cardiac lesions. Echocardiography is used to diagnose and quantify the degree of aortic stenosis. Neonates with critical aortic stenosis can present in congestive heart failure requiring urgent intervention, often a percutaneous balloon valvuloplasty. Infants, children, and adults are often asymptomatic, even in the setting of severe aortic stenosis. Surgical interventions for aortic stenosis include aortic valve repair, aortic valve replacement with a pulmonary autograft, or replacement with a mechanical valve. The Ross procedure describes using a pulmonary autograft in the left ventricular outflow tract and pulmonary homograft in the right ventricular outflow tract while the Konno procedure widens a stenotic left ventricular outflow tract. Aortic regurgitation and, less commonly, pulmonary homograft dysfunction can be seen after the Ross procedure.