The purpose of this paper is to describe all available evidence on the distinctive features of a group of 4 life-threatening acute aortic pathologies gathered under the name of acute aortic syndrome (AAS). The epidemiology, diagnostic strategy, and management of these patients has been updated. The authors propose a new and simple diagnostic algorithm to support clinical decision making in cases of suspected AAS, thereby minimizing diagnostic delays, misdiagnoses, and unnecessary advanced imaging. AAS-related entities are reviewed, and a guideline to avoid imaging misinterpretation is provided. Centralization of patients with AAS in high-volume centers with high-volume surgeons is key to improving clinical outcomes. Thus, the role of multidisciplinary teams, an “aorta code” (streamlined emergent care pathway), and aortic centers in the management of these patients is boosted. A tailored patient treatment approach for each of these acute aortic entities is needed, and as such has been summarized. Finally, a set of prevention measures against AAS is discussed.