Cardiac presentations of Marfan syndrome are not uncommon. Patients with Marfan syndrome can present with aortic pathology, valvular pathology, or both. Hence, Marfan syndrome patients can undergo more than one cardiac surgery in their lifetime. Endoscopic mitral valve surgery can be a good alternative to redo sternotomy. We present a case of a 77-year-old woman who had an aortic root replacement and replacement of the ascending aorta. She presented to our center 13 years later with severe mitral regurgitation. Femoral cannulation for cardiopulmonary bypass was not possible due to the presence of a type B aortic dissection that occurred sometime after the initial procedure. Mitral valve replacement was performed with an empty beating heart using axillary artery inflow with an excellent result.