A 26-year-old man was admitted for treatment of congestive heart failure resulting from aortic regurgitation. The patient had been on medical treatment for ulcerative colitis (UC) since he was 14 years old and for ankylosing spondylitis (AS) since he was 20 years old. On admission, gradients of blood pressure among the extremities were observed. Echocardiography revealed marked dilation of the left ventricle (LV), hypokinetic wall motion of the LV, slightly prolapsed aortic cusps with annular dilatation, and severe aortic regurgitation. Computed tomographic scans demonstrated an aneurysmal dilation of the ascending aorta and thickening of the descending and abdominal aortic wall. Digital subtraction angiography demonstrated an aneurysmal dilation of the ascending aorta; howeveir, there was no clear evidence of steno-occlusive lesions in the brachiocephalic vessels. Blood studies showed positive inflammatory signs and negative rheumatoid factor. HLA typing showed A2, 24(9), B27, 67, Cw1, 7, and DR1, 2. Based on these data, the diagnosis of Takayasu arteritis associated with UC and AS was made. Aortic root replacement was performed. Steroid therapy was restarted immediately after surgery. Histologic studies of the aortic wall showed findings compatible with Takayasu arteritis. The combination of these rare diseases suggests that they have a common pathophysio-logic background. (Jpn Circ J 1998; 62: 64 - 68)