Acquired hepatocerebral degeneration is an underdiagnosed neurologic syndrome characterized by parkinsonism, ataxia or other movement disorders and by neuropsychiatric and cognitive symptoms. It occurs in patients with chronic liver disease, especially those who develop portosystemic shunting and is often unrecognized as a cause of cognitive decline. Recently, its pathogenesis has been associated with manganese accumulation in basal ganglia and some treatments proposed. The aim of this article was to report a case and discuss some discoveries in connection with the disease.