A 45-year-old woman with an 8-year history of systemic lupus erythematosus (SLE) was admitted with complaints of sudden onset of chest and back pain and syncopal attack. Aortography showed DeBakey type I acute aortic dissection. She has been maintained on a small dose of corticosteroids (prednisone 5mg/day). After antihypertensive drug treatment, a replacement of the total aortic arch and arch vessels was successfully performed. The postoperative course was uneventful and she has had no relapse of SLE.