Both aortic valve commissural detachment and commissural fenestration are rare causes of aortic regurgitation. In general, aortic valve replacement is the mainstay treatment for aortic regurgitation caused by commissural detachment or commissural fenestration. We herein describe valve-sparing aortic root re-implantation and aortic valve repair for aortic regurgitation and aortic root dilatation in an extremely rare case accompanied by both commissural detachment and commissural fibrous strand of fenestrated cusp.