Creutzfeldt-Jakob disease (CJD) is a human prion disease with rapidly progressive neurodegeneration. The major clinical manifestations of CJD include mental deterioration, myoclonus, cerebellar dysfunction, and neuro-ophthalmic symptoms and signs. However, abnormal eye movements as an early sign of CJD are rare. We report a 49-year-old man with periodic alternating nystagmus in early disease course.