The history of a 67-year-old woman with histologically proven Creutzfeldt-Jakob disease (CJD) is presented. Before typical clinical and neurophysiological signs of CJD developed, magnetic resonance imaging (MRI) showed slightly enhanced signal intensity of the caudate nuclei and putamina in T2-weighted and proton density images, corresponding to spongiform degeneration in neuropathological examination. Five weeks later characteristical progressive cortical atrophy was demonstrated by follow-up MRI.