Rheumatic heart disease poses a major challenge to public health and is the most prevalent heart disease in children. The major determinants of rheumatic fever and rheumatic heart disease are poverty, malnutrition, overcrowding, poor housing, and a shortage of health-care resources. Although cost-effective strategies for the prevention and control of these diseases are available, they remain underutilized in most developing countries. A World Health Organization Expert Consultation reviewed the current scientific knowledge of rheumatic fever and rheumatic heart disease, as well as medical and public-health practices, and revised the Jones diagnostic criteria for rheumatic fever and rheumatic heart disease. This revision facilitates the diagnosis of: primary episodes of rheumatic fever, recurrent attacks of rheumatic fever in patients with or without rheumatic heart disease, rheumatic chorea, insidious onset rheumatic carditis and chronic 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.