Rheumatic fever resulting from group A β-hemolytic Streptococcus infection continues to be a serious cause of morbidity and mortality in developing countries. Guidelines for diagnosis and prevention of rheumatic fever are reviewed. A systemic review of literature was performed. Arthritis followed by carditis and chorea, are the main manifestations of disease. Evidence of asymptomatic carditis has been increasing; however, abnormality identified by echo-doppler evaluation is not considered as criterion for diagnosis of rheumatic fever. No single laboratory test can confirm diagnosis; however, some tests help to characterize inflammatory process and provide evidence of streptococcal infection. There are a lot of gray cases. Diagnosis is difficult even with Jones Criteria. Carditis and arthritis are signs of other systemic illness, although the history is different. In the acute stage, if no thorough history has been taken, signs of carditis and arthritis are minimal and will mislead patient to seek late medical advice. Therefore follow-up is needed as patients are seen in the general clinic. They should receive an early referral to a pediatrician with a multidisciplinary approach wherein a cardiologist could help to identify any problem. Benzathine penicillin continues to be the best therapeutic option for treatment and secondary prophylaxis, due to its efficiency and low cost.