Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are preventable diseases disproportionately affecting Aboriginal and/or Torres Strait Islander Australians living in remote areas. Prevalence rates were highest in the Northern Territory, females, and young people aged 5–14. These data highlight the important role of jurisdictional control programs and registers in Queensland, Western Australia, South Australia, the Northern Territory and New South Wales.