We describe our experience of surgical treatment in a patient with Takayasu's arteritis who required aortic root replacement because of perivalvular aortic regurgitation, developing 2 years after aortic valve replacement. A 65-year-old man underwent aortic valve replacement with a mechanical valve 3 years previously because of serious aortic insufficiency associated with Takayasu's arteritis. No steroids were given postoperatively. Three years after surgery, perivalvular aortic regurgitation developed. Reoperation was scheduled because of increased regurgitation and valve dehiscence. The sinus of Valsalva and the ascending aorta were enlarged, and a false aneurysm was found at the suture line of the aortotomy. Moderate mitral insufficiency was also present. The patient underwent aortic root replacement with a mechanical valve (J-graft Shield^®, 24 mm ; and SJM Regent^®, 21 mm), hemiarch replacement (J-graft Shield^®, 24 mm), and mitral annuloplasty (IMR ET Logix^® ring, 28 mm). Intraoperative examination showed very severe adhesion around the ascending aorta and marked wall thickening extending from the aortic root to the ascending aorta. The annulus was recognized to be very fragile after the mechanical valve was removed. The annulus was reinforced with autologous pericardium patch, furthermore, the subannulus was reinforced with a shortly cut artificial vessel graft. Aortic root replacement was then performed. After surgery, the patient received steroids. Inflammation was improved by steroids and the patient is being followed up on an outpatient basis. In patients with a fragile annulus and severe inflammation associated with aortitis, tissue reinforcement and postoperative management of inflammation are essential.