Prion diseases have recently emerged as a significant challenge to health-care workers, including those involved in dentistry. Abnormal prion proteins are resistant to complete inactivation by conventional sterilization techniques. In the last decade, a new form of prion disease emerged in the UK, termed "variant CJD", thought to be acquired by consumption of bovine spongiform encephalopathy-contaminated food products. At present, CJD is an invariably fatal disease with no immediate prospect of treatment or vaccination. Of concern with the variant form of CJD, unlike the more classic forms of the disease, is the appearance of significant levels of infectivity outside the central nervous system. This raises concerns for the potential transmission of prion proteins via surgical procedures from individuals in the asymptomatic stage of the disease. This article reviews the existing knowledge base on the nature of prions, their distribution in oral tissues, and the implications for dental treatment