We describe a case of Creutzfeldt-Jakob disease associated with a dura mater graft (Lyodura brand) in a 26-year-old man who underwent several neurosurgical procedures as a child. Clinicians and infection control personnel should be aware that recipients of Lyodura brand dura mater grafts processed before May 1987 may remain at increased risk for Creutzfeldt-Jakob disease throughout their lives.