The Ehlers-Danlos syndrome (EDs) is one of the most frequently encountered inherited disorders of connective tissue. The arterial-ecchymotic type IV is notorious for large vessel involvement associated with spontaneous catastrophic bleeding. Most of these patients who require cardiovascular surgical procedures have a poor prognosis. Our experience of two patients, with aneurysmal dilatation of the ascending aorta is presented. The first patient presented with an aortic dissection extending from the ascending aorta to the common iliac arteries. The second patient presented with asymptomatic but progressive aneurysmal dilatation of the aortic root. Both patients underwent the Bentall procedure using a 25 mm St. Jude composite valved conduit. Despite increased vascular friability, both of these patients tolerated the operative procedure without complication and are doing well on early follow-up. This experience suggests that the Bentall procedure may be used to decrease the risks of dissection and rupture of the ascending aorta in patients with Ehlers-Danlos syndrome.