Acute aortic syndromes (AAS) encompass a constellation of life-threatening medical conditions including classic acute aortic dissection (AAD), intramural haematoma, and penetrating atherosclerotic aortic ulcer. Given the non-specific symptoms and physical signs, a high clinical index of suspicion is necessary to detect the disease before irreversible lethal complications occur. In order to reduce the diagnostic time delay, a comprehensive flowchart for decision-making based on pre-test sensitivity of AAS has been designed by the European Society of Cardiology guidelines on aortic diseases and should be thus applied in the emergency scenario. When the definitive diagnosis is made, prompt and appropriate therapeutic interventions should be undertaken if indicated by a highly specialized aortic team. Urgent surgery for AAD involving the ascending aorta (Type A) and medical therapy alone for AAD not involving the ascending aorta (Type B) are typically recommended. In complicated Type B AAD, thoracic endovascular aortic repair (TEVAR) is generally indicated. On the other hand, in uncomplicated Type B AAD, pre-emptive TEVAR rather than medical therapy alone to prevent late complications, while intuitive, requires further study in randomized cohorts. Finally, it should be highlighted that there is an urgent need to increase awareness of AAS worldwide, including dedicated education/prevention programmes, and to improve diagnostic and therapeutic strategies, outcomes, and lifelong surveillance.