Farmers around the world face and manage a wide range of enterprise-related risks. These risks are increasing due to a range of factors including globalisation, increased trade in agricultural products, and climate change, jeopardising agricultural enterprises and forcing farmers to adjust their production and management strategies. Here we present results of a systematic literature review, following PRISMA protocol, of farmers’ perceptions of, and responses to, agricultural risks. Using data reduction method (factor analysis) and descriptive statistics, we analysed 197 studies and found that weather-related risk (55%), biosecurity threats (48%), and human risk (35%) are the significant risks perceived by farmers for their agricultural enterprises. Diversification of crop and animal production (28%) and pests and diseases monitoring and prevention (20%) were the preferred agricultural risk management strategies employed by farmers. Few studies have investigated socio-economic factors that explain risk perceptions (18%) or factors that influence how farmers manage agricultural risks (11%). The main barriers to successfully managing agricultural risks were limited access to information and formal low-interest loan systems, especially in developing countries. We identified a mismatch between perceived risk sources and risk management strategies, highlighting a need to improve understanding of why particular management responses are employed to address the various risks. This review suggests areas for future research to improve understanding of the perceptions of risks held by farmers, and to support efforts to manage and reduce these risks.