Acute aortic syndrome (AAS) is a life-threatening disease. Quick and accurate diagnosis is crucial. Patients should be transferred to a competence center without any delay as soon as AAS is suspected. Immediate onset of tearing chest pain, mediastinal widening on chest radiography and pulse/blood pressure differentials are predictive for aortic dissection. A CT scan is the diagnostic tool of choice; alternatively, in hemodynamically unstable patients echocardiography may be preferred. Associated mortality is excessively high within the first few days. Urgent surgical consultation should be obtained for all patients presenting with AAS. Initial medical therapy is aimed to reduce pain and decrease wall stress in the aorta. Aortic dissection involving the ascending aorta should be treated by immediate surgery. Aortic dissection limited to the descending or thoracoabdominal aorta should be treated medically, initially. However, when associated with complications, endovascular treatment is recommended. A symptomatic intramural hematoma, a penetrating atherosclerotic ulcer or pending aortic rupture are associated with a substantial risk. Therefore, surgical or endovascular therapy is recommended.