A 23-year-old male patient complained dyspnea on exertion and orthopnea since December 1977. On examination, he was tall and slender. There was grade IV/VI to-and-fro murmur on the left sternal border especially on Erb`s point. The liver was descended 2 fingers breadth below right costal margin. There were no signs of Marfan`s syndrome. Echocardiography demonstrated partial closure of aortic valve and dilated aortic root with enlargement of ascending aorta. Left heart cardiac catheterization revealed moderately elevated pulmonary wedge pressure and right ventricular pressure. The left ventricular end diastolic pressure was markedly elevated to 26 mmHg. On aortography, the aortic regurgitation was severe and it was belonged to angiographically Grade IV. The aortic valve was replaced with Carpentier-Edwards valve without excision and replacement of ascending aorta, under the impression of rheumatic valvular heart disease. After closure of aortotomy, blood pressure was transiently elevated and bleeding from the site of inserting air vent needle of ascending aorta was developed. The bleeding was not controlled by any means. On postmortem microscopic study, the histologic changes were strikingly limited to the ascending aorta from the region of the aortic valve ring.[KTCS 1979;3:183-190]