AIM: Acquired hepatocerebral degeneration (AHD) is an exceptional type of hepatic encephalopathies (HE). It is characterized by neuropsychiatric and extrapyramidal symptomathology similar to that seen in hepatolenticular degeneration (Wilsons disease). In this paper, we report a case of AHD with unusual presenting features.METHODS: A 28-year-old man with AHD was described and the literature was reviewed.RESULTS: The man had a history of HBV-related liver cirrhosis. He was admitted to our hospital with apathy,dysarthria, mild consciousness impairment and extrapyramidal symptoms after hematemesis. By review of the literature,cases with AHD often did not present consciousness impairment. So our case was once diagnosed incorrectly as Wilsons disease.CONCLUSION: AHD is a rare syndrome and its variable clinical manifestations make it difficult to be diagnosed.But we believe that extensive examination and thorough understanding of the disease are beneficial to a correct diagnosis. Moreover, biocoene is effective in treating the case.