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Introduction: The increasing prevalence of obesity and type 2 diabetes mellitus has become a global healthcare concern spreading to indigenous Arctic populations. As non-alcoholic fatty liver disease (NAFLD) is strongly associated with the metabolic syndrome, it has become a leading cause of chronic liver disease. However, data are sparse on the prevalence of NAFLD in indigenous Arctic populations who may have a different risk profile for diabetes complications., Methods: We conducted a systematic review to estimate the prevalence of NAFLD or signs of NAFLD in indigenous Arctic people inhabiting Greenland, Alaska, Canadian territories and Eastern Russia. Also, we wanted to discuss how Arctic research in metabolic disease such as NAFLD may move forward., Results: Through the pre-specified search of Ovid MEDLINE and Embase, 3,070 unique references were identified and six studies including 5,487 persons qualified for data extraction. The prevalence of NAFLD or signs of NAFLD varied between 21% and 65%. The risk of bias was considerable particularly due to the inclusion of small and heterogeneous studies., Conclusion: Only limited published research exists on NAFLD in indigenous Arctic populations. This review reports that the prevalence of NAFLD or signs of NAFLD in the indigenous Arctic populations residing in Arctic Regions may be similar to the global level, emphasising the need for further health research in indigenous Arctic populations., (Articles published in the DMJ are “open access”. This means that the articles are distributed under the terms of the Creative Commons Attribution Non-commercial License, which permits any non-commercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.)