Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of chronic, progressive, and fatal neurodegenerative disorders that affect a variety of mammalian species. This chapter discusses the issues raised by two foodborne prion diseases, namely bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD), particularly those related to their spread in cattle and humans, the contamination of specified risk material (SRM) in meat, the relevant regulations, and appropriate detection methods for surveillance.