Aortic disease is a leading cause of morbidity and mortality in Marfan syndrome, both in the adults and children. Sequelae include congestive heart failure due to aortic regurgitation, endocarditis, aortic dissection, and sudden death due to aortic rupture. While aneurysms in Marfan syndrome can occur in any aortic segment and may also involve the major branch vessels, the aortic root is particularly predisposed. Recent advances in the diagnosis and the medical and surgical management of the aortic abnormalities of the Marfan syndrome have achieved a dramatic improvement in the quality of life and the life expectancy of affected individuals. Key features of the therapeutic protocol include reduction of aortic wall stress through the use of beta adrenergic antagonists, prevention of bacterial endocarditis, and early and serial applications of diagnostic imaging techniques to aid in the timing of prophylactic surgical intervention. The surgical repair of aneurysmal aortic dilatation or DeBakey type III aortic dissection is indicated in patients with symptoms indicative of impending rupture, ischemic complications related to dissection, or an aortic ratio of 2.0. Proximal aortic dissection requires aortic root replacement. In most cases, a composite valve graft is used to replace the aortic valve and ascending aortic segment. Biologic valve grafts may be particularly suitable for selected populations of pediatric patients. Postoperative management should include appropriate antibiotic prophylaxis, continued therapy with β-adrenergic antagonists, and diligent surveillance for new or recurrent aortic disease in both repaired and unrepaired segments. In combination, these therapies clearly diminish morbidity and contribute to a prolonged survival in the Marfan syndrome.