Sporadic Creutzfeldt-Jacob disease (CJD) is a rare fatal neurodegenerative prion disease. Clinical findings of CJD consist of a rapid onset dementia, myoclonus, and neurologic disorders. Definitive diagnosis is established with histopathologic confirmation of brain parenchyma or autopsy materials. Periodic triphasic electroencephalography (EEG) changes and detection of 14-3-3 protein in cerebrospinal fluid are subsequent diagnostic criteria. A case of a 63-year-old female with painful rigidity, noncooperation, disorientation and bilateral postural tremor is reported. She had hypoactive deep tendon reflexes, moderate agitation, and drowsiness without any facial asymmetry. Her speech was extremely dysarthric and unintelligible, along with akinetic mutism. This case of sporadic CJD is presented with magnetic resonance imaging, EEG results, clinical history and laboratory findings.