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Bicuspid aortic valve (BAV) is known to cause the heart's aortic valve narrow. This prevents the aortic valve from opening fully causing reduction and/or block flow of blood from the heart. The clinical presentation of BAV is highly variable. In many cases, BAV is asymptomatic, however in some it may become calcified later in life. Its major manifestations are aortopathy, valvular dysfunctions and endocarditis. We report a case of BAV with a follow-up for over 40 years to assess the temporal association and progression of various complications for better understanding of disease process.