Creutzfeldt-Jakob Disease (CJD) is an infectious, progressive, degenerative neurological disorder. Unique CJD Precautions must be adhered to as the infectious agent is difficult to destroy. A regional group in Hamilton-Burlington Ontario developed CJD guidelines based on critical review of the current evidence of transmission in a Canadian healthcare environment, current published standards of practice in North America, the United Kingdom and Australia; and principles of laboratory and patient care safety as well as expert opinion.