Rheumatic heart disease (RHD), an autoimmune reaction to an infection of rheumatogenic group A streptococcus bacteria, is characterised primarily by progressive and permanent heart valvular lesions, although other parts of the heart may be affected. Despite an overall decrease in the incidence of RHD in developed countries, it remains a pertinent health issue with high rates in developing countries and amongst certain Indigenous populations in industrialised countries. Primary, secondary and tertiary strategies for the prevention of rheumatic heart disease exist, as do numerous barriers to such strategies. A review of the literature, incorporating its epidemiology and pathophysiology, demonstrates that interventions at various stages of the disease may reduce the collective burden of disease.