Following recent published evidence regarding the experimental transmission of prion diseases via blood transfusion, dental practitioners have expressed their concern about the potential impact of these transmissible spongiform encephalopathies on dental care provision. This review provides updated information on Creutzfeldt-Jakob disease and related disorders and highlights their potential significance for the practice of clinical dentistry. The current guidelines in Australia relating to infection control and clinical dental procedures are discussed together with recommended guidelines and considerations from the United Kingdom and the World Health Organization (WHO).