Preservation and restoration of the aortic valve and root, as well as relief of the stenosis, is of paramount importance during surgical treatment of supravalvular aortic stenosis. A 23-year-old woman with a history of medically treated endocarditis presented with progressive fatigue and dyspnea on exertion. A diagnostic work-up revealed multiple stenoses and a small saccular aneurysm involving the diffusely hypoplastic ascending aorta, in addition to severe mitral regurgitation. The aortic root and ascending aorta were replaced using the Yacoub remodeling technique. Hemiarch replacement was performed under hypothermic circulatory arrest, and the mitral valve replaced. By using a remodeling technique, it was possible to augment the left sinus of Valsalva, to obtain the largest diameter at the sinotubular junction, and to adjust the spatial relationship of commissures that renders good leaflet coaptation. Although extended aortoplasty has been used widely for supravalvular aortic stenosis, the Yacoub procedure is a good option in diffuse type hypoplasia with multiple stenosis of the ascending aorta.