Rheumatic fever and rheumatic heart disease remain a significant cause of cardiovascular morbidity and mortality in countries around the globe. Acute rheumatic fever (ARF) is a multisystem disease that occurs 2–4 weeks after infection with group A b-haemolytic streptococcus (GABHS). Mechanisms explaining the pathogenesis of rheumatic fever include Immune response, Genetic markers, Organism factors and Environmental factors as well as a rule of Virus. Essentials of diagnosis of rheumatic fever are the fulfillment of the Jones criteria lastly updated in 1992 which are the basis of WHO criteria 2002-2003. New diagnostic techniques for rheumatic carditis include: echocardiography, endomyocardial biopsy and radionuclide imaging as well as other new markers. Recent studies analyzed and compared different lines of treatment of acute rheumatic fever including surgey. Recommendations for primary and secondary prevention of acute rheumatic fever as well as prevention of infective endocarditis are continuously updated. The outcome of an acute attack varies from complete recovery to death from intractable heart failure.