A 26-year-old man with relapsing polychondritis was admitted for the treatment of multiple thoracic aortic aneurysms in the ascending and descending aorta. Descending thoracic aortic aneurysm showed rapid expansion, therefore, the patient underwent an extended thoracic aortic repair from the ascending aorta to the descending aorta via anterolateral thoracotomy and partial sternotomy. Although postoperative course was uneventful, aortic root enlargement and severe aortic insufficiency progressed over the next two years. He and his family refused redo surgical intervention and the patient died of heart failure. Careful perioperative follow-up may be mandatory in a patient with relapsing polychondritis complicated by cardiovascular disease.