In spite of much analysis of the impact of bovine spongiform encephalopathy (BSE) on consumer perceptions and meat purchases, there has been little explicit analysis of the level of BSE knowledge. In this study the role of knowledge about BSE was examined in Canada, the United States, and Japan. In addition, the level of knowledge was linked to human health concerns regarding BSE and whether there is agreement with paying a premium for beef with BSE animal tests. From a public policy perspective, understanding whether higher or lower knowledge is linked to public concern and desire for market intervention might help in the design of risk communication in any future animal disease outbreak. Should lack of knowledge about the disease be related to a public desire for market intervention (animal testing, for example), then an increase in detailed information about how humans might contract the disease might change public pressure for intervention. As compared to U.S. and Canadian respondents, Japanese respondents are more knowledgeable regarding the ways in which humans might be exposed to the human variant of BSE (variant Creutzfeldt-Jakob disease, vCJD) and are more concerned about the disease. However, U.S. respondents are more willing to pay a premium for beef tested to ensure that it will not result in vCJD. Japanese respondents who are more knowledgeable about BSE are more concerned about the risk of BSE to human health. In Canada, subjects who are more knowledgeable about the ways in which humans attain vCJD are less concerned about the risk of BSE to human health. Knowledge of the ways in which humans develop vCJD does not significantly influence concerns about the risk of BSE to human health in the United States or willingness to pay for BSE-tested beef in any of the three countries. The links between knowledge and concerns about BSE and between knowledge and agreement with paying premiums for BSE-tested beef were estimated for each country using ordered probit regressions.