This paper reviews scientific and gray literature addressing climate change vulnerability and adaptation in the Inuvialuit Settlement Region (ISR) in the western Canadian Arctic. The review is structured using a vulnerability framework, and 420 documents related directly or indirectly to climate change are analyzed to provide insights on the current state of knowledge on climate change vulnerability in the ISR as a basis for supporting future research and long-term adaptation planning in the region. The literature documents evidence of climate change in the ISR which is compromising food security and health status, limiting transportation access and travel routes to hunting grounds, and damaging municipal infrastructure. Adaptations are being employed to manage changing conditions; however, many of the adaptations being undertaken are short term, ad-hoc, and reactive in nature. Limited long-term strategic planning for climate change is being undertaken. Current climate change risks are expected to continue in the future with further implications for communities but less is known about the adaptive capacity of communities. This review identifies the importance of targeted vulnerability research that works closely with community members and decision makers to understand the interactions between current and projected climate change and the factors which condition vulnerability and influence adaptation. Research gaps are identified, and recommendations for advancing adaptation planning are outlined.