The bicuspid aortic valve (BAV) is the most common congenital cardiac malformation. A literature search was performed using the key words ‘bicuspid aortic valve’, ‘pathophysiology’, ‘exercise’ and ‘training’. BAV is the result of a complex developmental process where several genes seem to lead to abnormal valvulogenesis. Complications associated with BAV include aortic stenosis (AS) and regurgitation, infective endocarditis and aortic dilation and dissection. Moreover, BAV may be associated with other cardiovascular anomalies, mainly aortic coarctation. There is greater awareness of BAV in the young population who practice sport, with an increasing interest on the impact of regular and competitive exercise on athletes with BAV. The early identification of BAV through pre-participation screening is of paramount importance, and the justification of the more appropriate diagnostic methods is still an area of debate. A normally functioning BAV usually does not represent a limit for practising sport. The stress of regular and intense exercise on an abnormal aortic valve may favour its early deterioration and accelerate the development of complications. Therefore, athletes with BAV warrant regular follow-up, which should include echocardiographic assessment at least every year. The eligibility for participation and ability to continue to practise competitive sports in athletes with BAV cannot be generalized, but needs to be individualized depending on age, severity of lesions and type of sport. Further studies are required to elucidate the impact of physical training and competitive sports on the natural course of the BAV.