Rheumatic heart disease causes more than 200,000 deaths worldwide annually, with the vast majority of these deaths occurring in developing countries, yet there are few autopsy studies of rheumatic heart disease in these countries. We performed a retrospective review of 6218 autopsies performed during the period from 1990 through 2006, searching for cases of rheumatic heart disease based upon the macroscopic pathologic examination of the heart. We found 147 cases (2.4%) of rheumatic heart disease. There was an apparent increase in the number of cases in the past 5 years. There were 95 deaths that were directly attributable to rheumatic heart disease, with congestive cardiac failure being the most common cause of death in 75 cases. The mean age at death due to rheumatic heart disease was 38 years. There were more cases of rheumatic heart disease in Indigenous Fijians than Indo-Fijians, with an adjusted relative risk of 1.26 (95% confidence intervals from 0.87 to 1.86). Our findings reflect the high burden and early age of death due to rheumatic heart disease in Fiji and the Pacific region generally, and underline the need for early detection and adequate secondary penicillin prophylaxis in this region.