Rheumatic fever and rheumatic heart disease continue unabated in most of the developing nations, affecting young individuals. Focal outbreaks of smaller magnitude have also been reported since mid 1980s from industrialized western nations, where this disease had almost disappeared. Introduction of penicillin in mid 1940s had markedly changed the natural history of rheumatic fever, although the incidence of rheumatic fever declined in developed nations before that, due to better living conditions. Treatment of rheumatic fever chiefly involves use of antibiotics (penicillin) to eradicate streptococci, and anti-inflammatory drugs like salicylates or corticosteroids. Patients with severe carditis, congestive heart failure and/or pericarditis are best treated with corticosteroids as these are more potent anti-inflammatory agents than salicylates. Salicylates may be sufficient for cases with mild or no carditis. The treatment must be continued for 12 weeks. Several studies have shown that valvular regurgitation, and not myocarditis, is the cause of congestive heart failure in active rheumatic carditis. Therefore surgery with mitral valve replacement or repair is indicated in cases with intractable hemodynamics due to mitral regurgitation. Development of chronic valvular lesion after an episode of rheumatic fever is dependent upon presence or absence of carditis in the previous attack and compliance with secondary prophylaxis. Recurrences due to inadequate penicillin prophylaxis are responsible for hemodynamically significant chronic valvular lesions requiring surgery.