Coastal areas of the Arctic and Subarctic are vulnerable to accidental oil spills, impacting the ecosystem, society, and economy. This article provides a comprehensive overview of oil spill pollution issues in cold regions, focusing on environmental impacts, oil transport and fate, coastal/shoreline response measures, and the state of current international policies and regulations. Numerous studies have described the potential effects of oil pollution (crude oil and refined products) on wildlife (invertebrates, fish, birds, and marine mammals) and coastal communities within the Arctic and Subarctic regions. The observed detrimental effects are influenced by the oil fate and transport processes, including physiochemical attenuation and biodegradation, natural dissolution/dispersion following point-source release (surface and subsurface), entrainment by sea ice, and stranding onto shorelines (in which the residual oil may be translocated). Measures such as natural attenuation, bioremediation, manual removal, in situ burning, and washing/flooding are available for spill response in coastal regions. Case studies in cold regions are illustrated for a better analysis of practical response methods, implying that shoreline cleanup operations in the Arctic and Subarctic are more challenging than those in more temperate and populated regions because of environmental and logistical challenges. Regarding preparedness, a number of national and international policies, regulations, and guidelines have been established to advance oil spill prevention and response measures within the Arctic and Subarctic regions. Based on the state of knowledge presented in this review, recommendations are made for future research on oil spill pollution in coastal regions of the Arctic and Subarctic.