Acute aortic syndrome, an acute lesion of the aortic wall involving the media, comprises aortic dissection, intramural hematoma and penetrating ulcer. Recent advances in imaging techniques have helped us to understand the natural history and dynamics of this medical condition. Despite considerable advances in surgical treatment, the current high mortality rate in the acute phase can still be reduced by early clinical suspicion and improved surgical expertise. The advent and incorporation of endovascular treatment has opened up new perspectives in the management of this disease, and may improve the long-term prognosis. This article aims to review our current understanding of the natural history and therapeutic management of acute aortic syndrome.