Acute rheumatic fever (ARF) is an inflammatory disease of childhood that occurs following untreated group A streptococcal pharyngeal infections. Although it is well established that group A streptococcus is the sole etiologic agent of ARF, the pathogenesis and immune mechanisms are still not completely understood. In the pediatric population, ARF is the most common cause of acquired heart disease worldwide. This multi-systemic disorder can affect the heart, joints, skin and with the exception of the central nervous system, usually develops within 3 weeks after the preceding acute group A streptococcal pharyngitis. ARF remains highly prevalent in developing nations where crowding, poor hygiene and limited access to health care persist. Acute rheumatic fever and chronic rheumatic heart disease (RHD) are the most common rheumatic disease in children and young adults and remains a major public health problem in the developing countries, with high occurrence, while in developed countries the occurrence is very low. ARF is still the predominant form of morbidity among children in the developing countries.