Bicuspid aortic valve (BAV) is the most common congenital heart defect, affecting 0.5–1.5% of the population. It functions normally in infancy but may lead to complications such as ascending aorta dilatation, calcification, stenosis, infective endocarditis, dissection, and sudden death. BAV syndrome affects both the valve and aortic wall, with aortic changes linked to embryological defects involving neurocrestal cells. Historical insights trace back to Leonardo da Vinci, who observed BAV’s impaired function. Over time, researchers like Osler, Abbott, and McKusick highlighted its associations with aortic complications, coarctation, and increased risk of dissection. Hemodynamic studies demonstrated abnormal flow patterns in BAV, causing traumatic stress and potential aortic dilatation. Recent studies confirmed the congenital nature of BAV syndrome, emphasizing its multifactorial impact on cardiovascular health.