Disease prioritization is motivated by the need to ensure that limited resources are targeted at the most important problems to achieve the greatest benefit in improving and maintaining human and animal health. Studies have prioritized a range of disease types, for example, zoonotic and foodborne diseases, using a range of criteria that describe potential disease impacts. This review describes the progression of disease prioritization methodology from ad hoc techniques to decision science methods (including multi-criteria decision analysis, conjoint analysis and probabilistic inversion), and describes how these methods aid defensible resource allocation. We discuss decision science in the context of disease prioritization to then review the development of disease prioritization studies. Structuring the prioritization and assessing decision-makers’ preferences through value trade-offs between criteria within the decision context are identified as key factors that ensure transparency and reproducibility. Future directions for disease prioritization include the development of validation techniques, guidelines for model selection and neuroeconomics to gain a deeper understanding of decision-making.