The diagnosis of Creutzfeldt-Jakob disease (CJD) can be challenging as findings are non-specific and there is low awareness of the disease. We present a case of an 83-yearold man with a two months history of rapidly progressive dementia. After a series of extensive diagnostic examinations, he was diagnosed with probable sporadic CJD with key findings of rapidly progressive dementia, myoclonus, pyramidal signs, abnormal hyperintensity signals on diffusion-weighted magnetic resonance imaging (DW-MRI) and typical electroencephalograph (EEG). His symptoms progressively worsened and he died four months after the onset of symptoms.