Although substantial progress has been made in the prevention, diagnosis, and treatment of acute aortic dissection, it remains a complex cardiovascular event, with a high immediate mortality and substantial morbidity in individuals surviving the acute period. The past decade has allowed a leap forward in understanding the pathophysiology of this disease; the existing classifications have been challenged, and the scientific community moves towards a nomenclature that is likely to unify the current definitions according to morphology and function. The most important pathophysiological pathway, namely the location and extension of the initial intimal tear, which causes a disruption of the media layer of the aortic wall, together with the size of the affected aortic segments, determines whether the patient should undergo emergency surgery, an endovascular intervention, or receive optimal medical treatment. The scientific evidence for the management and follow-up of acute aortic dissection continues to evolve. This Seminar provides a clinically relevant overview of potential prevention, diagnosis, and management of acute aortic dissection, which is the most severe acute aortic syndrome.