Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are preventable diseases affecting socioeconomically disadvantaged populations globally, including Australian children. This study aims to describe the clinical presentation and outcomes of ARF and RHD in children aged less than 5 years, to improve recognition and management.A retrospective audit was undertaken of children aged less than 5 years with ARF and RHD in the Northern Territory (NT) of Australia between 2010 and 2020. Patients were identified from the NT RHD register. Descriptive data analyses were performed to summarise patient demographics, clinical presentation, diagnosis, disease severity, management, and outcomes.During the study period, 82 children (51% female) aged less than 5 years presented with definite, probable, or possible ARF (78/82), and/or RHD (20/82). Aboriginal and Torres Strait Islander children were disproportionately affected, with average annual incidences (2010-2020) of ARF and RHD of 127 (95% confidence interval [CI]: 99-155) and 33 (95% CI: 18-47) per 100,000 population, respectively. ARF recurrence was common (23%). At diagnosis, RHD was moderate to severe in 55% of cases, and 20% of children with RHD required one or more cardiac surgeries.Children aged less than 5 years living in remote NT are at risk of ARF and RHD. Aboriginal and Torres Strait Islander children are disproportionately affected. Presentations with sore joints, chorea, new cardiac murmurs or other symptoms suggestive of ARF should be fully investigated. Echocardiographic screening for RHD should be considered for children living in remote NT from the age of 3 years.