The concept of an acute aortic syndrome (AAS) facilitates early identification of patients with chest pain due to an aortic cause, and expedites implementation of definitive treatment. Important differentials are acute coronary syndromes and pulmonary embolism. Patients with AAS should be evaluated using a combination of blood tests, electrocardiography, and imaging, such as computed tomography, echocardiography, and magnetic resonance imaging. Aortic dissection is the most common form of AAS. Risk factors include congenital factors such as connective tissue disease and bicuspid aortic valve, and acquired disease such as hypertension. Patients with evidence of aortic rupture or end-organ ischaemia require urgent intervention, which in cases of type A dissection is typically open surgery and in type B dissection, endovascular stent graft placement. The optimal treatment 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 design individualized, patient-centred treatment.