Clinical features at onset of Creutzfeldt-Jakob disease (CJD) may mimic symptoms of Lewy bodies dementia. Clinical evolution, neuroimaging, metabolism exploration, and cerebrospinal fluid investigations may help establishing the diagnosis. However, CJD definite diagnosis requires postmortem autopsy. This symptom overlap led us to successfully prescribe an anticholinesterasic treatment, rivastigmine, to a patient for whom a probable CJD disease was finally diagnosed.