Rheumatic heart disease poses a major challenge to public health and is the most prevalent heart disease in children. A WHO Study group reviewed the current scientific knowledge of rheumatic fever and rheumatic heart disease as well as the medical and public health related practices and revised the Jones diagnostic criteria for rheumatic fever and rheumatic heart disease. The present report provides practical recommendations for an evidence-based approach to the prevention and management of rheumatic fever and rheumatic heart disease. It also provides practical guidance for implementing cost-effective programmes for controlling these diseases. The report will be of interest to clinicians policy-makers and public health professionals.