Wilson's disease is an autosomal recessive hereditary disease in which the capacity of biliary copper excretion is reduced, resulting in a toxic accumulation of this metal in the liver, brain and other organs. The neuroimaging techniques, computed tomography (CT) and nuclear magnetic resonance (NMR), have been incorporated to the diagnostic workup in patients with suspected Wilson's disease (WD). We report two patients with WD in whom CT and NMR were carried out for the evaluation of the central nervous system (CNS). The lesions appeared as hypodense areas in CT or signal abnormalities in NMR over the involved structures: putamen, caudate nucleus, cerebellar dental nucleus, red nucleus and subcortical white matter. In one of the patients, hypointense signal areas were found over both putamen nuclei in T-2 times of NMR, which might correspond to cavitary necrosis or copper deposition. The lesion distribution suggests that vascular lesions might play a role in the mechanisms of tissue damage. These findings show that CT and NMR are very helpful to evaluate WD. NMR images are quite characteristic of this disorder.