Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, affecting 1-2% of the general population. BAV is associated with an increased morbidity and mortality related to diseases of the aortic valve (aortic stenosis and aortic regurgitation) and the thoracic aorta (aortic aneurysm and aortic dissection). These conditions can often be treated surgically to prevent adverse events and reduce mortality. However, optimal management of BAV patients requires knowledge about the natural history of common clinical complications and involved pathological mechanisms. The aim of this article is to present common complications associated with BAV and to summarize the recently published evidence-based guidelines focusing solely on BAV patients.