BACKGROUND: Indigenous Australians experience high rates of chronic conditions. It is often asserted Indigenous Australians have low adherence to medication; however there has not been a comprehensive examination of the evidence. This systematic literature review presents data from studies of Indigenous Australians on adherence rates and identifies supporting factors and impediments from the perspective of health professionals and patients. METHODS: Search strategies were used to identify literature in electronic databases and websites. The following databases were searched: Scopus, Medline, CINAHL Plus, PsycINFO, Academic Search Premier, Cochrane Library, Trove, Indigenous Health infonet and Grey Lit.org . Articles in English, reporting original data on adherence to long-term, self-administered medicines in Australia's Indigenous populations were included. Data were extracted into a standard template and a quality assessment was undertaken. RESULTS: Forty-seven articles met inclusion criteria. Varied study methodologies prevented the use of meta-analysis. KEY FINDINGS: health professionals believe adherence is a significant problem for Indigenous Australians; however, adherence rates are rarely measured. Health professionals and patients often reported the same barriers and facilitators, providing a framework for improvement. CONCLUSIONS: There is no evidence that medication adherence amongst Indigenous Australians is lower than for the general population. Nevertheless, the heavy burden of morbidity and mortality faced by Indigenous Australians with chronic conditions could be alleviated by enhancing medication adherence. Some evidence supports strategies to improve adherence, including the use of dose administration aids. This evidence should be used by clinicians when prescribing, and to implement and evaluate programs using standard measures to quantify adherence, to drive improvement in health outcomes.