We report a 43-year-old patient of De Bakey type I aortic dissection [DA (I)] with a 2-year-history of systemic lupus erythematosus (SLE). He had had no treatment for SLE before the onset of dissection. Computed tomography (CT) on admission revealed DA (I), and he underwent emergency operation. Since the aortic valve and the left main coronary artery were severely damaged, aortic root replacement was performed. Coronary buttons were prepared in Carrel method and coronary reconstruction was performed in Piehler modification. After surgery, he suffered from repetitive hemolytic anemia. Corticosteroid therapy and rinsed blood transfusion were very effective for anemia. The combination with SLE and DA (I) was rare and this report of successful aortic root replacement is the second literature among English and Japanese papers.