The bicuspid aortic valve is one of the most common congenital anomalies. Its incidence is 0.9% to 2%. It may run in families with the affection of more than one family member. It may be associated with other congenital anomalies as coarctation of the aorta and patent ductus arteriosus. The most common complications of bicuspid aortic valve are aortic stenosis, aortic regurgitation, infective endocarditis and dissection. Bicuspid aortic valve is diagnosed mainly by two-dimensional echocardiography in the parasternal short axis.