Relapsing polychondritis (RP) is a rare, inflammatory, multiorgan disorder affecting cartilaginous structure and other connective tissue. Cardiovascular complications occur in 10%–52% of patients. We report a case of emergency surgery for an acute aortic dissection in a patient with RP. A 45-year-old female who had been taking corticosteroids (10 mg/day) for 2 years for RP presented at another hospital with dyspnea and severe chest and back pain. Acute aortic dissection was diagnosed, and we performed emergency replacement of the ascending aorta. We could not control the bleeding from suture holes of the native aorta because the vessel was so fragile. Thus, we performed a delayed sternal closure. The day after surgery, bleeding had decreased, and we could close the chest wall. Infection was well controlled, and the patient suffered minimal additional complications. She was discharged to home by herself 29 days after surgery and returned to normal life.