Bicuspid aortic valve (BAV) is the most common congenital heart disease with an estimated prevalence of 0.5-2%. BAV is more commonly observed in males with approximate predominance of 3:1. A bicuspid aortic valve results from partial or complete fusion of two of the aortic valve coronary cusps (left, right or non coronary). This results in an abnormal valve opening with either a horizontal or vertical raphe. There may be varying degrees of aortic stenosis or aortic regurgitation. Development of dilation and aneurysms in the adjacent aorta (aortic root, sinotubular junction and most commonly the ascending aorta) are not uncommon during adolescence and adulthood.