Creutzfeldt-Jakob disease is the most common form of human prion diseases. A 57-year-old woman was transferred to our Department from a local hospital, where she had been treated for two weeks due to consciousness disorders and convulsive epileptic attacks that progressed to refractory status epilepticus. Electroencephalography showed diffuse spike-wave complex discharges and development of nonconvulsive status epilepticus. The causes of metabolic encephalopathy and paraneoplastic syndrome were ruled out. A combination of clinical features and findings of diagnostic procedures including electroencephalography, biomarkers in the cerebrospinal fluid and magnetic resonance imaging suggested with great probability that the patient was affected with sporadic Creutzfeldt-Jakob disease.