We report a 70-year-old man who manifested with hemidystonia associated with focal status epilepticus. The subsequent clinical symptoms and signs including rapid progressive dementia, and generalized myoclonus and the presence of 14-3-3 protein in cerebrospinal fluid, typical magnetic resonance imaging and eletroencephalography findings provide the evidences for diagnosis of probable Creutzfeldt-Jakob disease (CJD). Movement disorders or epilepsy rarely occur in the early stage of CJD. Furthermore, co-occurrence of both disorders in the early stage has been hardly reported. To the best of the authors’ knowledge, this is the first report in Korea of CJD presenting as dystonia. Our case suggests that CJD can present with a variety of movement disorders.