We report in this article a girl with an initial diagnosis of autoimmune hepatitis who developed full-blown systemic lupus erythematosus (SLE) at her two-years follow-up. She was formerly considered as HBV-related chronic active hepatitis but due to the persistence of elevated liver enzymes, the reversal of the albumin and globulin ratio and abnormal HBV serology, she was later diagnosed as autoimmune hepatitis. With the clinical findings of arthritis, arthralgia and malar rash and supported by results of laboratory tests, she was diagnosed as a case of unusual SLE presenting with autoimmune hepatitis. We conclude, therefore, that each patient with a diagnosis of autoimmune hepatitis in childhood who exhibits abnormal HBV serology must be evaluated for a possible diagnosis of SLE.