Great Bear Lake, Northwest Territories, Canada supports important sport and aboriginal-subsistence fisheries and is among the last remaining pristine great lakes of the world. The lake’s unique ecology is characterized by a harsh subarctic climate, low productivity and species diversity, and high intraspecific diversity of lake trout. These aspects in combination with geographical remoteness present special challenges to the management of two exceptionally different fisheries. The history of its management has not been well documented or reviewed; therefore, our objectives in this paper were to summarize the history and status of Great Bear Lake’s fisheries and their management, and to identify gaps in knowledge, future challenges, and actions required to meet those challenges. Prior to 1970, management goals for the lake had not been established formally, and harvest numbers and biological characteristics of fish were unknown. To reduce data gaps, creel surveys, gillnet assessments, fish tagging, and subsistence monitoring were implemented. During the 1980s, Canada established the management goal of conserving a high quality sport fishery, while protecting aboriginal access to the subsistence fishery. On the basis of assessment data, lodge harvest quotas, lodge guest capacity limits, individual angler harvest limits, and angler licensing were among the management actions taken to achieve that goal. Since 2005, decision making has been guided by the “water heart,” a management plan for Great Bear Lake. Management has since evolved into a complex co-management system among aboriginal, territorial, and federal governments. Changes in regulations, sport trophy and tourism industries, subsistence resource use, and social and cultural norms and practices contributed to changes in the Great Bear Lake fishery and its management. In the future, anthropogenic and climate change are the two main challenges facing co-management of the lake’s resources. We recommend the adoption of an ecosystem approach to management, establishment of a fishery technical committee, reformulation of the current plan, explicit commitment to evaluation, conducting community-based monitoring, and development and use of a joint strategic plan among co-managers to describe how to interact and implement the Great Bear Lake management plan.