Streptococcus pyogenes (Group A Streptococcus) causes a variety of diseases, from benign self-limiting infections of the skin or throat to lethal infections of soft tissue accompanied by multi-organ failure. GAS is one of significant species among Gram-positive pathogens which is responsible for several suppurative infections and non-suppurative sequelae. They also cause pharyngitis, streptococcal toxic shock syndrome (STSS), necrotizing fasciitis and other diseases. Currently, global burden of RF / RHD is undervalued. In 2010, RF and RHD were estimated as 15.6 million cases and deaths around 200,000 annually. Laboratory diagnosis includes cultural techniques, serology, PYR test, Bacitracin susceptibility test and antibiotic resistance testing helps in differentiating the Streptococcus pyogenes from other groups of Streptococci. Most of the Acute Rheumatic Fever cases gets missed or does not present in the initial stage rather it has been developed into advanced Rheumatic Heart Disease condition. Modified Jones criteria in 2015 will be helpful especially to the low risk population as it is challenging because of limited access to primary health care, diagnosis of streptococcal disease. In addition to this revised criteria, diagnosis still relies on clinical diagnostic algorithm. Vaccines based on M protein and T antigens are continuing to evolve with different results. Ongoing vaccine development is still challenging for the GAS research community, it will make a positive and lasting impact on the peoples globally.