Sjögren’s syndrome (SS) can shows various neurologic symptoms as it involves the central, peripheral and autonomic nervous system. However, CNS involvement as a initial manifestation is rare. We report a 21-year old woman who presented with vertigo, gait ataxia, and hiccup lasted about one month due to a discrete dosal medullary lesion in primary SS. The symptoms and lesions improved by the intravenous methylprednisolone therapy. Key Words: Sjogren’s Syndrome; Nystagmus, Physiologic; Nervous System Diseases