A 39-year-old woman with Marfan's syndrome was referred with a symptom of exertional dyspnea, had mitral valve regurgitation, annuloaortic ectasia with aortic valve regurgitation and Stanford B type chronic aortic dissection. She was successfully treated with a one-stage operation, consisting of aortic root replacement with the Carrel patch method, mitral valve replacement and extended replacement of the thoracic aorta (ascending, arch and thoracic descending aorta), through median sternotomy and left antero-axillary thoracotomy. This operation was performed under hypothermic circulatory arrest with continuous retrograde cerebral perfusion. The postoperative course was uneventful. Although the operation may include complicated procedures, it is important to perform a sufficient operation corresponding to the patient's condition and lesions, employing the most advanced surgical techniques, such as circulatory arrest, myocardial protection and so on.