We report a patient with Creutzfeldt-Jakob disease with somnolence as an initial manifestation and sleep apneas as a prominent sign. A respiratory polygraphy revealed the presence of a continuous Cheyne-Stokes pattern both awake and during sleep with central apneas. The neuropathology showed PrP deposition in brainstem involving respiratory nuclei. Central apnea is a rare manifestation of CJD that can be observed from early stages and may be related with PrP deposition in brainstem structures.