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Quadricuspid aortic valve (QAV) is a rare congenital anomaly frequently associated with other anomalies particularly coronary anomalies. It may be detected on transthoracic or transesophageal echocardiography. We present here a case report of a 27-year-old male patient with a QAV, the valve being regurgitant and requiring aortic valve replacement. It has been reported as isolated case reports in the literature and various theories exist to the development of QAV. The diagnosis requires a high degree of suspicion and a detailed assessment, and if asymptomatic, then patients need to be carefully followed up for the development of aortic regurgitation.