The transmissible spongiform encephalopathies (TSEs) represent an emerging group of diseases that have been labeled as “prion diseases” because of the recent characterization of the infectious agent. TSEs are caused by prions, which induce neurodegenerative fatal diseases in humans and animals. Some TSEs (scrapie and kuru), have existed in both animals and humans for a very long time, whereas others such as bovine spongiform encephalopathy and variant Creutzfeld–Jakob disease have either recently emerged or are more thoroughly described and recognized. It is obvious that the medical community will be forced to consider these diseases in humans and animals for the future. This article offers a short review of the TSEs of immediate concern to zoo and wildlife veterinarians and wildlife biologists and suggests risk management strategies for the prevention of these diseases, with special focus on chronic wasting disease of cervids in North America.