The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) was established in October 1993 after the identification of probable iatrogenic CJD in recipients of human pituitary hormones. Since this time and with the recommendations of the Allars inquiry into CJD in Australia, the registry has performed surveillance of CJD in Australia with retrospective ascertainment to 1970 and ongoing prospective ascertainment of all human prion diseases or transmissible spongiform encephalopathies (TSEs). Prion diseases include CJD, Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia and Kuru. This brief summary presents the epidemiological findings of the ANCJDR based on data from 1970 to 31 December, 2004.