Aortopathy associated with bicuspid aortic valve (BAV) encompasses a spectrum of morbid entities, including ascending aorta dilatation, coarctation, and dissection. BAV-related aortopathy, first noted by Abbott in 1928, stems from structural weaknesses in the aortic wall, such as cystic medial necrosis with mucoid extracellular matrix accumulation and elastic fragmentation. Even normally functioning BAV predisposes to progressive aortic dilatation, with growth rates of 1–2 mm annually, occasionally exceeding 5 mm/year and necessitating preventive surgical intervention. Histopathological studies link aortic wall abnormalities of BAV to deficiencies in smooth muscle cells and extracellular matrix components, compounded by genetic predispositions and hemodynamic stress. Pregnancy further exacerbates dissection risks due to immune-related alterations of connective tissues. Preventive strategies, including echocardiographic monitoring and prophylactic aorta replacement at threshold diameters (5.0–5.5 cm), are fundamental. Pharmacological therapies like β-blockers and angiotensin receptor blockers prove promising in handling BAV-associated aortopathy by mitigating aortic dilatation, akin to Marfan syndrome. Understanding the pathophysiological mechanisms and risk factors underlying BAV aortopathy is crucial for tailored management strategies, early detection, and prevention of life-threatening complications like aortic dissection and rupture.