In this report, we describe the case of a 28-yr-old woman with sickle cell anemia who presented with acute hepatic failure manifested by anorexia, malaise, painless jaundice, elevated aminotransferase activities, and severe coagulopathy. Liver biopsy revealed changes consistent with autoimmune hepatitis. Treatment with corticosteroids and azathioprine was followed by improvement in biochemical liver test results. The literature on sickle cell-associated liver diseases is reviewed.

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