Logopenic aphasia, mainly characterized by word anomia, sentence and phrase comprehension difficulties secondary to phonological loop deficits but relatively preserved single word comprehension and no agrammatism, is one of the 3 main variants of primary progressive aphasia (PPA). We describe the first case of PPA that fulfilled clinical criteria of logopenic aphasia but showed abnormal DWI hyperintensities that were predominant on the left hemisphere and compatible with Creutzfeldt-Jakob disease (CJD). After abnormally long isolated language deficits, the patient rapidly worsened and died. Autopsy performed 18 months after onset of language difficulties confirmed the diagnosis. We therefore advocate performing DWI sequences in any suspicion of PPA in order to rule out CJD.