A patient with histopathologically verified sporadic Creutzfeldt-Jakob disease (CJD) presented initially with diplopia, sleep disturbances, and L-dopa-responsive parkinsonism. After more than a year of slow progression, he did not become demented, and failed to fulfill the clinical criteria for possible CJD. No clinical examinations currently proposed to detect CJD showed the disease. CJD should be in the differential diagnosis of "parkinson plus" syndromes until a different etiology has been found or a histopathologic examination performed.