The bicuspid aortic valve is the most frequent congenital anomaly of the heart in adults. Since the 1970s, with the advent of two-dimensional echocardiography, the in-vivo diagnosis of bicuspid aortic valve has become easier and greater attention has been paid to this anomaly in normal young people, particularly in sportsmen because, from a theoretical point of view, physical exercise may stress the abnormal aortic valve, favouring an early deterioration of the valve itself, as well as a dilation of the ascending aorta. Although the bicuspid aortic valve may remain without clinical consequences for a lifetime, it may be responsible for severe complications such as aortic stenosis, aortic insufficiency, endocarditis and aortic dilation/dissection, sometimes requiring surgery. Moreover, the bicuspid aortic valve may be associated with other cardiovascular anomalies, mainly aortic coarctation. At present there are no prospective studies dealing with effect of physical training and competitive sports on the natural course of the bicuspid aortic valve. However, in order to take any decision about sports eligibility, sports physicians should perform an initial accurate staging of the bicuspid aortic valve, taking into account haemodynamic factors, aortic complications and associated significant cardiovascular anomalies. A strict follow-up, with serial cardiological controls, is mandatory as well as antibiotic prophylaxis for endocarditis, particularly in subjects engaged in contact sports.