Quadricuspid aortic valve (QAV) is a rare congenital anomaly with <200 cases reported till date. Most patients are asymptomatic. Symptoms depend on functionality of the valve and associated heart defects, most often presenting as aortic regurgitation followed by aortic stenosis. Management guidelines are ill-defined. We present a case series of 2 patients of QAV with rare associations and variable clinical presentation.