Acquired (non-Wilsonian) hepatocerebral degeneration (AHD) is a rare irreversible neurologic syndrome that occurs in patients with chronic liver disease associated with multiple metabolic insults. The pathophysiology and the locations of the cerebral injuries are incompletely understood. We describe a patient with fatal hepatic cirrhosis and AHD in whom MR images showed abnormalities in the brachium pontis bilaterally. Neuropathologic evaluation disclosed multiple regions of subcortical spongiform white matter changes.