Aortic regurgitation (AR) and, aortic root dilatation are serious problems occurring after arterial switch operation (ASO). We have experienced a case of 12-year-old boy with aortic root dilatation and AR after ASO. Consistent progression of AR and aortic root dilatation were observed during the follow-up period. The latest Z-score of Valsalva sinus was about 9. We performed the Bentall procedure instead of aortic valve-sparing operation because of the degenerative aortic valve and the excessive aortic root dilatation. The postoperative course was uneventful. Histopathological findings of the aortic wall showed degeneration of elastic fibers on both sides of the anastmosis.