A 51-year-old woman was admitted to our hospital because of systemic jaundice, general fatigue in August 24, 2007. She was diagnosed with systemic lupus erythematosus (SLE) as a result of a discoid rash, photosensitivity, lymphocytopenia, elevated serum anti ds-DNA antibody and a positive test for antinuclear antibody. Her laboratory data revealed severe liver dysfunction, suggesting autoimmune hepatitis (AIH). She was also diagnosed with thrombotic thrombocytic purpura (TTP) because of thrombocytopenia, hemolytic anemia, renal dysfunction and decreased ADAM-TS13 activity. The patient was treated by methylprednisolone pulse therapy, fresh frozen plasma infusion and ursodeoxycholic acid. Her symptoms and laboratory data rapidly improved and a liver biopsy was carried out. Interface hepatitis and lymphocyte infiltration were observed in the specimen. A diagnosis of definite AIH was made from her International AIH group score of 20 points. AIH and TTP are rare complications of SLE. The prevalence of the complication of SLE and AIH has been reported as 1.7 approximately 2.7%, and that of SLE and TTP as 1 approximately 4%. We reported here a rare case of SLE complicated with AIH and TTP.