By the mid 1980s, bovine spongiform encephalopathy (BSE) emerged in the United Kingdom (UK) and reached its peak in the early 1990s with up to 37,000 cases. In the year 2000, BSE was diagnosed for the first time for a cow born in Germany. Since then, 413 cases of BSE have been detected. About 10 years after the first BSE cases were detected, variant Creutzfeldt-Jakob disease (vCJD), a new variant of Creutzfeldt-Jakob disease (CJD), was described in the UK. Legal measures for protection from BSE are described. The number of cases of vCJD and the development of the BSE situation in Germany and Bavaria until 2009 are presented.