An update on animal spongiform encephalopathies other than BSE was presented in part I of this article. Part II of this article focuses on bovine spongiform encephalopathy (BSE). The article describes clinical signs, histological and other changes leading to a diagnosis, the differential diagnosis, and the prospects for pre‐clinical diagnosis of this disease. It describes the origin and course of the epidemic in the UK and discusses the question why it is only the UK which has witnessed such a large‐scale epidemic. Predicted to arrive much earlier, the epidemic now finally seems to have passed its peak. The article goes on to discuss the chance of the disease occurring in a country like the Netherlands and the measures taken to prevent and control the disease, should it occur.