Several pathogenetic mechanisms account for the association of the ascending aorta dilation with aortic valve dysfunction. Functional aortic insufficiency can derive from medial degeneration of the aortic wall and annuloaortic ectasia; leaflet structural disease can determine root dilation by increasing aortic wall stress in case of both regurgitation and stenosis; aortic valve disease and aortic aneurysm can however coexist due to two different intrinsic etiologies. In the attempt to best tailor the surgical correction of such conditions to the underlying causative mechanism, several technical options have already been developed including composite or separate aortic valve and root replacement, valve-sparing operations, and aortoplasty techniques. The criteria for surgical indication cannot leave the underlying pathogenesis out of consideration as well. The newly acquired knowledge in the basic research on this topic is expected to affect the approach to the individual patient in the future.