The majority of ascending aortic aneurysms cannot be related to any specific etiology and should be qualified as idiopathic. The pathobiology of ascending aortic aneurysms remains incompletely understood. Data from direct study are still scarce and often limited because of patient heterogenicity. Currently available information suggests that destructive remodeling of the aortic wall, inflammation and angiogenesis, biomechanical wall stress, and molecular genetics are relevant mechanisms of idiopathic ascending aortic aneurysm formation and progression. Further understanding of these mechanisms will likely provide novel diagnostic, prognostic, and therapeutical tools for the clinician.