We experienced a surgical case of aortic dissection (Stanford type A) with annuloaortic ectasia in Marfan Syndrome. A 45-year-old female who had been diagnosed as Marfan Syndrome three years age was emergently admitted to our hospital with sudden chest pain. We diagnosed this case as aortic dissection of Stanford type A by ultrasonic cardiogram and thoracic CT. The ascending aorta was replaced with composite graft by modified Bentall procedure, right coronary artery was bypass to segment 2 using vein graft and left coronary artery was interposed by Dacron graft. During operation we utilized retrograde cold blood cardioplegia for cardiac arrest (180 min.) and retrograde cerebral perfusion accompanied with total circulatory arrest (45 min.). No complication such as cardiac damage and cerebral damage was found postoperatively. We discussed surgical method for aortic dissection with anuloaortic ectasia in particular reconstruction of coronary artery, cardioplegia and cerebral protection.