Sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disorder of unknown etiology that causes rapidly progressive dementia. This disease is uniformly fatal and most patients die within 12 months. Clinical findings include myoclonus, visual disturbances, and cerebellar and pyramidal/extrapyramidal signs in addition to rapidly progressive cognitive and functional impairment. These findings are all non–specific and it is often difficult and challenging to diagnose premortem because of low awareness and clinical suspicion. Here, we present the case of a 65 year old female with a 1.5 month history of rapidly progressive dementia. After a series of extensive diagnostic examinations, she was diagnosed with probable sporadic Creutzfeldt–Jacob Disease as per the Centres for Disease Control and Prevention (CDC) criteria, with key findings of rapidly progressive dementia, with visual disturbances along with hallucinations, extrapyramidal signs (cogwheel rigidity), and abnormal hyperintensity signals on diffusion–weighted MRI. Her symptoms progressively worsened and the patient was eventually lost on follow–up. The clinicians should be aware of the existence of such a disease in India and should have an index of suspicion for such disease when a patient presents with above spectrum of symptoms