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Indigenous Peoples in the Arctic have for millennia relied on freshwaters for drinking water and freshwater species that comprise important subsistence harvests, which promotes a strong connection to the land and unique understanding of organisms and ecosystem processes and changes. Despite the importance of freshwater biodiversity and ecosystem services to Arctic Indigenous communities, there have been limited attempts to summarise available Indigenous Knowledge (IK) regarding Arctic freshwater systems and to understand how conservation can benefit from this knowledge base.This paper presents a systematic review of literature documenting circumpolar Arctic IK with a focus on freshwater biodiversity in Canada, Greenland, Fennoscandia (Norway, Sweden, and Finland), Russia, and the U.S.A. (Alaska). Standardised search terms and methodologies were used to locate relevant documents using Google Scholar and Google Advanced search engines. Thematic coding was used to identify freshwater biodiversity themes within the identified documents.Documented IK of freshwater biodiversity was found from all five geographic regions and included data on both species presence and habitat changes with potential to affect biodiversity. Canada had the highest number of relevant documents (n = 127), followed by the U.S.A. (Alaska; n = 116), Fennoscandia (n = 38), Russia (n = 27), and Greenland (n = 5). The number of relevant documents with IK published per year was highest in most recent years, from 2010 onwards, in all geographic regions.Fish represented the highest number of faunal observations with 59 species observed, approximately half of which were Salmonidae (29 species). Local‐scale assessment of fish diversity found observations of the highest number of species (11–25) in Alaska, and individual observations of 6–10 species were found throughout Alaska, mainland areas of Canada, and the Kola Peninsula in Russia. Documented IK also contributed new information on historical fish diversity and indicated local‐scale loss or gain of species. Such information is of vital importance to provide long‐term records of fish composition and abundance, especially when this information does not exist in other knowledge bases such as western science datasets.Indigenous Knowledge included observations of changes in freshwater and terrestrial habitat associated with a warming climate, such as: decreasing water levels and more draining/drying of lakes and rivers, a shorter period of ice cover (late freeze and early break‐up), decreasing ice thickness, and increasing occurrence of permafrost thaw and eroding banks. Such observations by those who actively rely on Arctic freshwater ecosystem services are important because they signify that change is occurring and that action is needed to mitigate the impacts on freshwater habitats and the biodiversity therein.This study demonstrates that previously documented IK provides valuable information towards determining freshwater biodiversity baselines and patterns of change in the circumpolar Arctic. However, these results do not sufficiently cover the depth and breadth of IK on freshwater biodiversity and ecology held by Indigenous communities. Further work incorporating Indigenous worldviews around freshwater ecology would provide context to the knowledge collected and a deeper understanding of Arctic circumpolar freshwater environments. [ABSTRACT FROM AUTHOR]