AbstractBackgroundIndigenous Australians are at increased risk of developing dementia – Alzheimer disease and mixed dementia diagnoses are the most common. While prion diseases have been reported in Indigenous peoples of Papua New Guinea and the United States, the occurrence and phenotype of prion disease in Indigenous Australians is hitherto unreported.AimTo report the incidence rate and clinical phenotype of Creutzfeldt‐Jakob disease (CJD) in Indigenous Australians.MethodCrude sporadic CJD (sCJD) incidence rates and indirect age standardisation of all CJD were assessed to calculate the standardised mortality ratio (SMR) of the Indigenous Australian population in comparison to the all‐resident Australian population, along with analysis of clinical phenotypes.ResultsWe report an illustrative case of an Indigenous Australian from regionally remote Western Australia dying from typical 'probable' sCJD 2 months after disease onset, with Australian National CJD Registry (ANCJDR) surveillance overall demonstrating eight Indigenous Australians dying from sCJD (five post‐mortem confirmed, three classified as 'probable') with a clinical phenotype similar to non‐indigenous people, including median age at death of 61 years (interquartile range IQR = 16 years) and median duration of illness of 3 months (IQR = 1.6 months). Indigenous Australians with sCJD were geographically dispersed throughout Australia. The calculated overall crude annual rate of sCJD in Indigenous Australians compared to the remainder of the Australian population was not significantly different (0–3.87/million for Indigenous Australians; 0.94–1.83/million for non‐indigenous). The overall indirect age‐standardised CJD mortality ratio for the indigenous population for the years 2006–2018 was 1.49 (95% CI, 0.75–2.98), also not significantly different to the all‐resident Australian population.ConclusionCJD occurs in Indigenous Australians with clinical phenotype and occurrence rates similar to non‐Indigenous Australians. These findings contrast with a previous report where the incidence rate of CJD in a non‐Australian indigenous population was reported to be decreased.