© 2015 Wolters Kluwer Health, Inc. PURPOSE OF REVIEW: To describe new developments (2013-2014) in acute rheumatic fever (ARF) and rheumatic heart disease (RHD) relevant to developing countries. RECENT FINDINGS: Improved opportunities for the primary prevention of ARF now exist, because of point-of-care antigen tests for Streptococcus pyogenes, and clinical decision rules which inform management of pharyngitis without requiring culture results. There is optimism that a vaccine, providing protection against many ARF-causing S. pyogenes strains, may be available in coming years. Collaborative approaches to RHD control, including World Heart Federation initiatives and the development of registers, offer promise for better control of this disease. New data on RHD-associated costs provide persuasive arguments for better government-level investment in primary and secondary prevention. There is expanding knowledge of potential biomarkers and immunological profiles which characterize ARF/RHD, and genetic mutations conferring ARF/RHD risk, but as yet no new diagnostic testing strategy is ready for clinical application. SUMMARY: Reduction in the disease burden and national costs of ARF and RHD are major priorities. New initiatives in the primary and secondary prevention of ARF/RHD, novel developments in pathogenesis and biomarker research and steady progress in vaccine development, are all causes for optimism for improving control of ARF/RHD, which affect the poorest of the poor.