Acquired hepatocerebral degeneration (AHD) is a neurologic syndrome occurring in the presence of chronic hepatic disease, which results in permanent neurologic aberrations. The syndrome shares many of the clinical manifestations of Wilson disease, however, distinction can be made with slit lamp examination and biochemical studies. Cranial magnetic resonance (MR) imaging of a patient with AHD revealed increased signal intensity in the dentate nuclei bilaterally on T2-weighted images indistinguishable from Wilson disease. The MR findings of Wilson disease as described in the literature are not specific and further investigation with slit lamp examination and biochemical studies is warranted to distinguish between the two entities.