Creutzfeldt-Jakob disease (CJD) is presumably caused by a slow infectious pathogen or prion. The principal clinical features of Creutzfeldt-Jakob disease are dementia, pyramidal and extrapyramidal symptoms and signs, cerebellar dysfunction, and myoclonus. The patient rapidly deteriorates, declines to a vegetative state, becomes comatous, and is ultimately dead within several months. The authors present a case of Creutzfeldt-Jakob disease, proved by clinical findings, typical serial EEG, and pathologic features.