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The risk of zoonotic disease transmission from animals to humans is elevated for people in close contact with domestic and wild animals. About three-quarters of all known human infectious diseases are zoonotic, and potential health impacts of these diseases are higher where infectious disease surveillance and access to health care and public health services are limited. This is especially the case for remote circumarctic regions, where drivers for endemic, emerging, and re-emerging zoonotic diseases include anthropogenic influences, such as pollution by long-range transport of industrial chemicals, climate change, loss of biodiversity and ecosystem alterations. In addition to these, indirect effects including natural changes in food web dynamics, appearance of invasive species and thawing permafrost also affect the risk of zoonotic disease spill-over. In other words, the Arctic represents a changing world where pollution, loss of biodiversity and habitat, and maritime activity are likely driving forward occurrence of infectious diseases. As a broad international consortium with a wide range of expertise, we here describe a selection of case studies highlighting the importance of a One Health approach to zoonoses in the circumarctic, encompassing human health, animal health, and environmental health aspects. The cases highlight critical gaps in monitoring and current knowledge, focusing on environmental stressors and lifestyle factors, and they are examples of current occurrences in the Arctic that inform on critically needed actions to prepare us for the future. Through these presentations, we recommend measures to enhance awareness and management of existing and emerging zoonoses with epidemic and pandemic potential while also focusing on the impacts of various environmental stressors and lifestyle factors on zoonoses in the Arctic.