The diagnosis of probable Creutzfeldt-Jakob disease (CJD) requires compatible clinical manifestations, typical electroencephalographical findings, and 14.3.3 protein positive in cerebrospinal fluid. Actually, MRI findings are not necessary, but they may support this diagnosis. The authors report a patient with definitive diagnosis of CJD who showed in a first MRI study performed two years before the clinical onset of the disease the same hyperintensities in caudate nuclei that were found in the last MRI. The authors think that these findings could be useful in detecting asymptomatic patients who need more extensive study and following.