The concept of acute aortic syndrome (AAS) facilitates the early identification of patients with chest pain caused by an aortic condition, and expedites risk stratification and definitive treatment. Important differential diagnoses are acute coronary syndrome, pulmonary embolism and chest pain from trauma. Individuals with AAS should be evaluated using a combination of blood tests, electrocardiography and imaging, such as computed tomography CT or echocardiography initially, and magnetic resonance imaging at a later stage. Classic aortic dissection is the most common form of AAS. Risk factors include congenital factors such as hereditary connective tissue disorders, bicuspid aortic valve with aortopathy and, most importantly, acquired conditions such as untreated hypertension. Patients with evidence of rupture of one aortic layer, malperfusion syndrome or end-organ ischaemia require urgent intervention; in cases of proximal dissection including the arch this is typically open surgery, and in distal or type B dissection it is usually endovascular stent-graft placement with ancillary procedures. The optimal management of patients with AAS is challenging and requires a multidisciplinary team approach. Further studies are required to fully characterize conditions within the AAS spectrum and to offer an individualized, patient-centred treatment.