Creutzfeld –Jacob Disease (CJD) is a rare type of neurodegenerative disease caused due to prion disease and is rapidly progressive and fatal. It is usually suspected based on the typical presenting symptoms, most important symptom being rapidly progressive dementia. Cases with atypical presentation are usually missed due to low clinical suspicion. We present a case report of a 64–year–old male who presented with worsening cerebellar ataxia and only mild dementia.MRI done to rule out any organic cause, revealed classic findings of CJD.Following this EEG was performed. MRI helped in clinching the diagnosis in this case of low clinical suspicion. This was an extremely rare variant of CJD–the brownell openheimer variant.(1)