Bicuspid aortic valve is the most common form of congenital valvular disease, and its presence may predispose the patients to development of true aortic or dissecting aortic aneurysms. An intrinsic aortic weakness may underlie a bicuspid aortic valve, aortic aneurysm or aortic dissection. We experienced a case of an ascending aortic aneurysm associated with a functionally normal bicuspid aortic valve. A screening echocardiograph was performed due to atypical chest pain, which detected a large ascending aortic aneurysm and a bicuspid aortic valve. The patient underwent an operation for an ascending aortic aneurysm and, is living one year later, and is asymptomatic. To the best of our knowledge, this may be the first case of an ascending aortic aneurysm associated with a bicuspid aortic valve in Korea.