The patient with thoracic aortic aneurysm disease requires careful evaluation and management over his or her lifetime. This includes assessment for the presence of an underlying genetic disorder, such as Marfan syndrome, bicuspid aortic valve disease, or a familial aortic aneurysm syndrome. Screening family members is necessary, inasmuch as up to 20% of first-degree relatives of the patient with a thoracic aortic aneurysm will also have aneurysm disease. Medical therapy is often prescribed, and beta-blocker therapy to reduce the stress on the aortic wall is usually recommended. However, very few clinical trials of pharmacologic therapy in humans with thoracic aortic aneurysm disease have been conducted. Mouse models have led to important discoveries and insight into the pathogenesis of aneurysm syndromes, and there is hope these may lead to effective therapy in people. Several studies are ongoing that examine the role of angiotensin receptor blockers in Marfan syndrome. Lifestyle modification is also important for patients with thoracic aortic aneurysm, including restrictions on physical activity, weight lifting, and recommendations about the management of pregnancy. Long-term surveillance of the aorta, even after successful surgery, is necessary for timing of prophylactic surgery and to evaluate for late complications.