The incidence of bicuspid aortic valve is 1-2%, making it the most common congenital cardiovascular anomaly. It often occurs in multiple members of the same family, suggesting autosomal dominance. It has a male predominance and it is associated with coartation of the aorta. The most common complication of bicuspid aortic valve is aortic stenosis and severe stenosis is a frequent indication for aortic valve replacement in young patients (<60 years of age). The features of aortic stenosis associated with a bicuspid aortic valve are similar to degenerative calcification of tricuspid aortic valves, although degeneration occurs at a much younger age. Bicuspid aortic valves are also at risk of developing aortic regurgitation and at risk of endocarditis