We report an unusual case of bicuspid aortic valve in a 67-year-old man who had aortic valve replacement for combined severe aortic stenosis with moderate aortic regurgitation. Both preoperative echocardiography and angiocardiography failed to recognize the bicuspid nature of the aortic valve, which was discovered only at surgical pathology examination. The atypical anatomic findings of the valve consisted of a "cleft-like" appearance of the ventral leaflet formed by the two conjoined cusps together with a fenestrated rudimental raphe.