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Primary Biliary Cirrhosis (PBC) and Autoimmune Hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC-AIH overlap syndrome. Acute hepatic failure is an unusual initial form of presentation of PBCAIH overlap syndrome. We report the case of a 31 year-old woman with autoimmune PBC-AIH overlap syndrome who presented with an acute hepatic failure. She revealed a good response to corticosteroid and ursodeoxycholic acid therapy.