Creutzfeldt-Jakob disease (CJD) is a neurodegenerative condition caused by prions. In literature there are distinguished four forms of CJD: sporadic (sCJD), familial, iatrogenic and variant (vCJD). Current diagnostic criteria of these forms, basing on clinical symptoms of this disease, allow to distinguish probable and possible CJD. Unquestionable diagnosis can be established only after the patient’s death on the basis of characteristic neuropathological picture. Intravitally, in CJD diagnosis the result of anatomopathological examination can be taken into consideration (cerebral biopsy, palatine tonsils). A case has been presented of a 53-year-old female in whom basically the only clinical symptom (for 45 days) was psychogenic blindness followed by coma. Then, she survived eight months in a vegetative state. The clinical symptoms of the disease fulfilled the criteria of variant CJD but neuropathological examination revealed sCJD. The establishing of the diagnosis in the case of CJD usually takes weeks and even months and the diagnosis of vCJD results in epidemiological and thus economic complication. The presented case was an atypical course of sCJD. Authors wanted to emphasize that sCJD starts not always with the onset of dementia but that it is possible that it starts with psychotic symptoms.