Creutzfeldt-Jakob's disease (CJD) is characterised by rapidly progressive dementia, ataxia, myoclonus, and several other neurological deficits. It generally affects older adults and occurs in sporadic, genetic and iatrogenic forms. Death occurs usually within one year after onset of the disease. The diagnosis is based on clinical criteria, neurophysiological and radiological findings, and confirmed by postmortal histopathology. During the last two years several cases of CJD have been reported with diffusion-weighted magnetic resonance imaging (MR) abnormalities, represented by increased signal intensity indicating reduced diffusion in basal ganglia and/or cortex cerebri. These abnormalities seem to be characteristic of CJD. We report a case of CJD in a 54-year-old woman who developed vertigo, nystagmus, ataxia, myoclonus, and dementia over a period of eight months. Diffusion-weighted magnetic resonance imaging showed increased signal intensity in corpus striatum and gyrus cinguli. The diagnosis was postmortally confirmed with histopathology.