Rheumatic fever is a multisystem inflammatory disease that occurs as a delayed sequelae to group A streptococcal pharyngitis. The important clinical manifestations are migratory polyarthritis, carditis, chorea, subcutaneous nodules and erythema marginatum occurring in varying combinations. The pathogenesis of this disorder remains elusive: an antigenic mimicry hypothesis best explains the affliction of various organ systems after a lag period following pharyngeal infection. In its classic milder form, the disorder is largely self-limited and resolves without sequelae, but carditis may be fatal in severe forms of the disease. Chronic and progressive damage to the heart valves leads to the most important public health manifestations of the disease. Anti-inflammatory agents provide dramatic clinical improvement, but do not prevent the subsequent development of rheumatic heart disease. The role of corticosteroids in treatment of carditis is uncertain and controlled studies have failed to demonstrate improved long term prognosis. Chorea, once considered a benign self-limited disease, is now felt to require more aggressive treatment, in particular with sedatives. Prevention of first and subsequent attacks of rheumatic fever is the mainstay in the limited arsenal available to alter the natural history of this disease.