Clinicopathologic correlative studies have shown that the corticobasal syndrome (CBS) is associated with various pathologies, including PSP, AD or sporadic Creutzfeldt-Jakob disease (sCJD), in addition to corticobasal degeneration (CBD. sCJD is generally distinguished from CBD by the much shorter duration of illness and the finding of either a 14-3-3 positive CSF test or periodic sharp wave complexes (PSWCs) in the EEG or a characteristic DWI-MRI signal. However, because of the existence of distinct disease subtypes, sCJD may show a less specific clinical course, which hampers the clinical distinction from CBD or other neurodegenerative disorders in case of overlapping clinical presentations. We observed a CBS in a subject with sCJD MV2, a disease subtype characterized by a relative long disease duration, the absence of EEG PSWCs, and a relatively high frequency of negative or inconclusive 14-3-3 CSF test.