Antimitochondrial antibody (AMA) is one of the representative features of primary biliary cirrhosis (PBC). PBC is a female-dominant disease usually presenting intrahepatic bile duct destruction, cholestasis and fibrosis with or without chronic nonsuppurative destructive cholangitis. We presented the case of a 63-year-old man with acute liver failure who had AMA, pronounced alanine aminotransferase elevation and high bilirubinemia. We administered corticosteroids and rescued this patient without liver transplantation. It is well known that some patients within the spectrum of autoimmune liver disease present with characteristics of both PBC and autoimmune hepatitis. Although corticosteroids may be associated with a significant worsening of adverse events in patients with PBC, if acute liver failure in AMA-positive cases is progressive, the administration of corticosteroids has to be considered, as well as the preparation of urgent liver transplantation.