Creutzfeldt-Jakob disease (CJD) is a rare type of prion disease leading to a rapidly progressive dementia (RPD) and other associated neurological features specially startle myoclonus. The annual incidence of CJD has been reported to be 1 per million or 271 people in USA2, but from Asia especially from Pakistan, only few case reports have been published. The probable cause behind the rarity of this disease in our society is still unclear.The diagnosis of CJD requires a high level of suspicion and lack of the availability of specific markers like CSF 14-3-3 in our setup makes it more difficult. Similarly brain biopsy is not an acceptable option for families of the sufferers because of the unavailability of the specific treatment. To our knowledge this is the first biopsy proven case of Crutzfeld Jacob disease (CJD) from Pakistan.