Creuzfeldt-Jakob disease belongs to a group of prion diseases characterised by rapid clinical deterioration and high mortality due to the lack of specific treatment. In approximately 85% of cases the disease is sporadic, however, both congenital and jatrogenic [1,2]. Despite widespread decades-long knowledge of the disease by doctors, especially neurologists, confirming the diagnosis remains exceptionally challenging. We present a 69-year-old patient who was admitted to a university hospital due to complaints of progressive gait and balance impairment and cognitive changes. Due to the clinical appearance and radiological examinations, Creuzfeldt-Jakob disease was suspected, and an electroencephalography and liquor tests carried out. The patient’s neurological and overall state worsened, and the liquor test results received postmortem confirmed the clinical and radiological diagnosis.