Fourteen patients with biochemically proven Wilson's disease underwent neuroimaging evaluation. The MRI manifestations could be classified as degenerative change and atrophic change. The degenerative change, most well documented on T2-weighted image, usually involves thalamus, lentiform nucleus midbrain, cerebellum, pens and caudate nucleus. The atrophic change, which is better shown on T1-weighted image, reveals as brain stem, cerebellar or cortical atrophy as well as ventricular dilatation. Repeated MRI evaluation was performed in nine patients who had received treatment for more than 14 months, degenerative change could be reversed significantly in eight cases and another one case whose MRI was kept in normal condition, which suggest the importance of long-term management.