Surveillance of all human prion diseases in Australia has been the responsibility of the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) on behalf of the Australian Government Department of Health and Ageing since the Registry's inception in October 1993. The ANCJDR was established in response to the identification of 4 CJD deaths in recipients of human-derived pituitary hormone. The initial brief was to perform focused surveillance for any further iatrogenic cases of CJD; however the scope of surveillance was soon expanded to include all cases of CJD occurring in Australia both prospectively and retrospectively to 1970. The activities of the ANCJDR have evolved from: routine surveillance responsibilities to detailed epidemiological analysis at both national and international levels; expert advice in relation to, and management of, infection control issues; and the provision of a number of tests to aid the diagnosis and classification of CJD in suspect cases. In this brief report, surveillance outcomes are examined with the inclusion of figures from the reporting period of 1 April 2009 to 31 March 2010 and the diagnostic services offered by the ANCJDR are outlined to provide a greater insight into this aspect of the Registry.