Rheumatic heart disease (RHD) caused by acute rheumatic fever (ARF) is a disease of poverty, poor hygiene and poor living standards. RHD remains one of the major causes of childhood cardiac disease in developing nations. Within developed nations, there has been a dramatic drop in the prevalence of RHD because of the improvement of living standards, access to health care and the widespread availability of penicillin-based drugs. Despite a dramatic reduction of RHD in Australia overall, it continues to be a major contributor to childhood and adult cardiac disease in Indigenous communities throughout northern and central Australia. Currently, Australia has among the highest recorded rates of ARF and RHD in the world. The most accurate epidemiological data in Australia come from the Northern Territory's RHD control programme. In the Northern Territory, 92% of people with RHD are Indigenous, of whom 85% live in remote communities and towns. The incidence of ARF is highest in 5–14-year-olds, ranging from 150 to 380 per 100 000. Prevalence rates of RHD since 2000 have steadily increased to almost 2% of the Indigenous population in the Northern Territory, 3.2% in those aged 35–44 years. Living in remote communities is a contributing factor to ARF/RHD as well as a major barrier for adequate follow-up and care. Impediments to ARF/RHD control include the paucity of specialist services, rapid turnover of health staff, lack of knowledge of ARF/RHD by health staff, patients and communities, and the high mobility of the Indigenous population. Fortunately, the recently announced National Rheumatic Fever Strategy, comprising recurrent funding to the Northern Territory, Queensland and Western Australia for control programmes, as well as the creation of a National Coordination Unit suggest that RHD control in Australia is now a tangible prospect. For the disease to be eradicated, Australia will have to address the underpinning determinants of poverty, social and living conditions.