Recent developments in our understanding of prion diseases have raised concerns for the public health. There is now compelling evidence that the transmissible agent for variant Creutzfeldt-Jakob disease (vCJD) in affected individuals is accumulated in lymphoreticular tissues such as the appendix and tonsils. This agent demonstrates a remarkable resistance to standard methods of sterilisation used in hospital sterile services departments. The possible implications this has on the safety of surgical instruments in ENT and other surgical practice is discussed. This review also outlines the history of our understanding of prion diseases and describes the development of a diagnostic test for vCJD in the living patient by pharyngeal tonsil biopsy.