Rheumatic heart disease is still a relevant problem in children, adolescents and young adults. Molecular mimicry between streptococcal and human proteins has been proposed as the triggering factor leading to autoimmunity and tissue damage in rheumatic heart disease. Despite the widespread application of Jones' criteria, carditis is either underdiagnosed or overdiagnosed. Endocarditis leading to mitral and/or aortic regurgitation influences morbidity and mortality of rheumatic heart disease, whilst myocarditis and pericarditis are less significant in determining adverse outcomes in the long-term. Strategy available for disease control remains mainly secondary prophylaxis with the long-acting penicillin G-benzathine.