A new phenotype of Creutzfeldt-Jakob disease termed new variant Creutzfeldt-Jakob disease (nvCJD) was first described in March 1996. This differs from other forms of CJD in terms of its epidemiology, clinical features and neuropathology. To date 24 cases of this new form of CJD have been described, 23 within the UK. This article describes nvCJD discussing clinical and epidemiological features and discusses possible links with the bovine spongiform encephalopathy epidemic in cattle in the UK.