Creutzfeldt-Jakob disease (CJD) is a rare prion disease that leads to a rapidly progressive dementia (RPD) and associated neurological features. It is not well documented in our country; therefore its true prevalence in Pakistan is not known. Here we report two cases of sporadic probable CJD seen in our hospital. The first, a 62 years old female, presented with RPD and myoclonus. The second was a 72 years old female who presented with generalized axial and limb rigidity, mutisim, personality changes and hallucinations along with RPD. Both cases were diagnosed as CJD on the basis of clinical, MRI and EEG findings.