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The unicuspid aortic valve is an extremely rare congenital anomaly. It usually presents with aortic stenosis and/or aortic regurgitation. Other cardiovascular complications, such as aortic dilatation and left ventricular hypertrophy can accompany it. Herein, we present a case report of a 50-year-old asymptomatic male patient with unicuspid aortic valve, complicated by ascending aortic aneurysm.