The bicuspid aortic valve (BAV) is the most common congenital anomaly of cardiac semilunar valves, mostly asymptomatic in youth but prone to complications such as stenosis, incompetence, infective endocarditis, aortopathy, aortic dissection, and sudden death; while mostly isolated, BAV can be associated with other congenital malformations, including atrioventricular and ventriculo-arterial connection anomalies, aortic arch coarctation, ventricular septal defects, and left outflow tract obstructions. Aortic arch obstruction is the most frequent associated anomaly (70% of cases). BAV is also linked to chromosomal disorders like Trisomy 18 and De George syndrome. Rare cases feature coexistence of bicuspid aortic and pulmonary valves. The etiology remains unclear, with potential roles of genetic, hemodynamic, and neural crest cell abnormalities.