Rheumatic fever and rheumatic heart disease continue to be a huge public-health burden on many Pacific Island countries. Prevalence reported in some nations are some of the highest seen globally, yet many countries in the region do not have national disease registers. Despite the will of many Pacific Island countries, there are a number of barriers to the implementation and sustainability of effective coordinated prevention programs, including limited funding and competing health priorities. In promising recent developments, a number of countries in the region have been able to develop or strengthen national rheumatic heart disease registers. These registers allow for more effective delivery of secondary prophylaxis, the mainstay of disease control in the Pacific. Primary prevention of rheumatic fever and screening for rheumatic heart disease are important adjunctive strategies. Recent advances in screening methods, focusing on portable echocardiography, may allow for the early detection of rheumatic heart disease in the community.