Given the growing proportion of elderly people in Western societies and the increasing prevalence of chronic hypertension, the management of aneurysmal aortic disease is an ever growing challenge. Although degenerative changes in the aortic wall are common to thoracic aortic aneurysm (TAA) and to various types of dissection in general, TAA can result from specific heritable disorders of connective tissues. Today, increased awareness of vascular diseases and access to tomographic imaging equipment facilitate the diagnosis of TAA, even when asymptomatic. While most TAA cases with ascending aortic involvement are treated with surgical repair (primarily valve-preserving techniques), aneurysms of the distal arch and descending thoracic aorta are amenable to alternatives to classic open repair such as the emerging endovascular treatment techniques. In this Review, we provide a comprehensive overview of the etiology, pathophysiology and clinical management of patients with TAA, and discuss the most recent literature on the condition.