Being considered among the most fatal neurological conditions, Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy characterized by its unknown etiology and rapidly progressive neurodegenerative symptoms that often lead to a mean survival of 6 to 12 months. The accumulation of the prionic protein causes brain matter degeneration, which leads to a set of clinical findings that include rapidly progressive dementia, myoclonus, tremors, cerebellar ataxia, and extrapyramidal signs. This clinical presentation is non-specific, which makes CJD a very difficult condition to diagnose, due to the low level of clinical suspicion. However, combining this clinical presentation with neuroimaging, a lumbar puncture and an encephalogram will help us make the correct diagnosis. We present the case of a 57-year-old male presenting to the Emergency department with complaint of personality change and intermittent memory loss. The patient's physical exam was significant for resting pill roll tremor, bilateral cogwheel rigidity, dysmetria, and shuffling gait. Magnetic resonance imaging of his brain showed symmetric bilateral diffusion signal abnormality involving the cortex, bilateral caudate heads and putamina. Continuous electroencephalogram revealed multiple bifrontal delta discharges with triphasic morphology. Lumbar puncture was significant for presence of 14–3-3 protein in cerebrospinal fluid. The multiple examinations performed in conjunction with the previous findings supported the diagnosis of acute encephalopathy secondary to sporadic CJD.