A 28-year-old woman presented with Marfan syndrome combined with severe mitral regurgitation and annuloaortic ectasia. The ascending aorta was dilated to 48 mm in diameter without aortic regurgitation. Considering the increased operative risk due to complication with aortic dissection, simultaneous replacement of the mitral valve and aortic root were performed. Her postoperative course was uneventful. Several options of the surgical treatment for Marfan syndrome are discussed.