Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and ultimately fatal disorder of the central nervous system. CJD occurs worldwide with an incidence of approximately .5-1 new cases per million population per year. Despite numerous epidemiological and laboratory studies, the etiologic agent and mode of transmission remain unclear. Clinical diagnosis is challenging and is based on clinical symptoms. No specific treatment is available and medical management remains limited to supportive care. Health care workers must be well informed about necessary precautions and decontamination procedures. Nurses play a major role in providing supportive care, educating community organizations and agencies about CJD and assisting the family through the grieving process.