Creutzfeldt-Jakob disease (CJD) is a progressive disease that is characterized by the accumulation of abnormal prion-like proteins in the central nervous system. The cerebral cortex is primarily affected in CJD, leading to spongiform changes and dementia. To date, there have been no reported cases of CJD, with local neuroparalysis discovered at an early stage of the disease. Here, we describe a patient who presented unilateral vocal cord and soft palate paralysis before the progression of CJD. After developing forgetfulness 6 months ago, a 76-year-old woman was presented at department of Otorhinolaryngology in a general hospital for recently developed hoarseness and dysphagia. In the oral and laryngeal endoscopic findings, unilateral paralysis of the vocal cord and soft palate was noted. On videofluorography, the larynx failed to elevate straight on swallowing. The right tongue pharyngeal wall was lax, and some contrast agent was retained in the lower right piriform sinus. The paralysis was thought to be due to the glossopharyngeal nerve or vagal nerve damage, which was caused by peripheral nerve injury or infranuclear palsy. Diffusion-weighted magnetic resonance imaging (MRI) revealed high signals in the cerebral cortical area (a signature feature of CJD). The patient died 2.5 years after the onset of illness. The patient was diagnosed as probable sporadic CJD. Since we could not detect any peripheral organic findings that could cause the paralysis, we suspect that CJD is responsible for the paralysis. In treating CJD patients with neurological signs, exclusive investigation is required to obtain a more detailed picture of the disease.