For the last decade, we have witnessed dramatic changes in both diagnostic and therapeutic aspects of acute aortic syndrome (AAS). With recent advances of various noninvasive aortic pathology imaging modalities, aortic intramural hematoma (AIH), a variant form of classic aortic dissection (AD), has emerged as an increasingly recognized and potentially fatal AAS entity. As the natural course of AD and AIH, especially with medical treatment, has been reported quite different, there has been a suggestion of applying different treatment strategies based not only on the affected sites of the aorta but also the different AAS entities, which is still in debate. Endovascular stent-graft placement is a revolutionary change in the treatment of various aortic pathologies including AAS. In the next decade, dramatic changes in clinical practice and outcome for patients with AAS are expected using new diagnostic information to select the best treatment option for individual patients.