A sinus of Valsalva aneurysm is a rare cardiac disorder, and reports of it with an anomalous origin of the coronary artery are scarce. A 35-year-old male was admitted to our department with fatigue and cough. Multi-detector-row computer tomography (MDCT) revealed an isolated extracardiac right sinus of Valsalva aneurysm with an anomalous origin of the left circumflex artery (LCX) and total occlusion of the right coronary artery (RCA). Its diameter was about 70 mm. We performed a partial aortic root remodeling procedure with a trimmed J-graft because he had neither aortic regurgitation (AR) nor annuloaortic ectasia (AAE). Concomitantly, coronary artery bypass grafting to the RCA (Seg. 3) using a saphenous vein, and reconstruction of the LCX by Piehler's technique using a saphenous vein were added. The patient's postoperative course was uneventful, and he was discharged on the 28th postoperative day. Postoperative MDCT revealed that the aneurysm of the right sinus of Valsalva was not enhanced, and the RCA and LCX were patent. This procedure preserved the patient's own normal aortic valve and sinus of Valsalva and enables him to have more physiologically normal hemodynamics than aortic root reconstruction using a composite graft, e.g. Bentall procedure, Cabrol procedure, although the potential progression of the AR requires careful follow-up.