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Progressive supranuclear palsy (PSP) is a clinical syndrome comprising vertical supranuclear palsy, parkinsonism,postural instability, and mild dementia. Other disorders can present with similar clinical pictures. In this report wedescribe a case of Creutzfeldt-Jakob disease (CJD) presenting with the PSP and discuss which features may help toprevent misdiagnosis. To the best of our knowledge, this is the first report of CJD presenting with PSP syndrome in Korea