Twenty-three patients with biochemically proved Wilson disease underwent magnetic resonance (MR) imaging of the brain. Positive findings, believed secondary to this condition, were found in 15 subjects. Findings varied among patients, but there were striking similarities between certain groups of patients. Areas of abnormal signal were seen in the lenticular, thalamic, caudate, and dentate nuclei, as well as in the brain stem; in these areas, the abnormalities were bilaterally symmetric. A smaller number of patients had asymmetric focal white-matter lesions. Correlation of the MR findings with clinical symptoms was generally good. Repeat imaging was performed on five patients at intervals ranging from 4 to 8 months; none showed significant interval change.