The aim of this article was to provide the dental community with a brief overview of the characteristics, risk of transmission, and the infection-control implications of prions in dentistry. MEDLINE, EMBASE, CINAHL, The Cochrane Library, and relevant databases were searched, and a targeted internet search was conducted up to July 2007. Transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative diseases that are rapidly progressive and always fatal, with no approved cure, and their definite diagnosis can only be obtained at post mortem autopsy. The causative agent, prion protein, resists conventional sterilization methods especially when infected tissue becomes dried onto glass or metal surfaces. To date, there are no reported definite or suspected cases of disease transmission arising from dental procedures, and there seems to be no correlation between dental treatment and TSEs. Because there is a theoretical but real risk of transmission of prion disease from dental instruments (although it is extremely low, especially in North America), as a general rule, appropriate family and medical history (including the risk for prion diseases) should be obtained from all patients, before all dental procedures. TSE research regarding diagnosis, transmission, treatment, and inactivation of prions and other transmissible amyloidoses are ongoing, and, thus, dental professionals should maintain optimal and up-to-date standards of knowledge, infection control, and decontamination.