Infective endocarditis (IE) of the bicuspid aortic valve (BAV) represents a severe life threatening clinical condition, occurring in the setting of bacteremia. BAV has become the most frequent predisposing factor for IE, exceeding mitral valve prolapse or rheumatic valve disease. The pathophysiology involves endothelial injury and fibrin deposition, providing a favorable milieu for microbial adhesion, predominantly gram-positive bacteria such as streptococci and staphylococci. Septic vegetative lesions can lead to valve perforation, systemic embolism, and severe complications like aortic aneurysm, fistulae, or conduction system involvement. BAV-IE is associated with a higher risk of perivalvular complications and neurological events, compared to tricuspid aortic valve (TAV) IE. While IE risk in BAV patients is historically estimated at 10–30%, recent studies suggest much lower rates, leading to new guidelines without routine antibiotic prophylaxis. However, clinical debate persists regarding prevention strategies. Advanced diagnostics, including blood cultures and echocardiographic imaging Duke criteria, are critical for early detection. Surgical intervention is recommended to remove large vegetations (>10 mm) and prevent embolism or in cases of severe valve destruction, to ensure better outcomes.