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A case of Creutzfeldt-Jakob Disease (CJD), a rare prion disease, posted for surgery is a clinically challenging scenario for an anesthesiologist. In addition to weighing in the consequences of using general versus neuraxial anesthesia for a patient of such a progressive neurodegenerative disorder, the anesthesiologist must also meticulously plan and institute a robust infection control protocol, keeping in mind that prion diseases are transmissible and highly resistant to the standard sterilization process. We hereby report the successful anesthetic management of a 57-year-old patient with suspected CJD presented with intertrochanteric fracture femur for closed reduction and internal fixation and also review the existing literature regarding the anesthetic management of a definite or suspected case of CJD.