Betahemolytic strains of streptococcus A are able to induce a spectrum of immunologically induced diseases, depending on the immunogenic M structure of the bacteria as well as on the genetic determined reaction of the host. In acute rheumatic fever (ARF) the Jones criteria, revised and modified several times and updated in 1992, remain the diagnostic standard. Echocardiography, still not included in the Jones criteria, has become a very important diagnostic tool, especially as half of the ARF induced carditis cases are clinically inapparent. Diagnosis may be very difficult if arthritis is the only major sign, especially if not occurring in the typical migrating pattern, a fact frequently reported from countries with a high risk of ARF. Poststreptococcal reactive arthritis (PSRA) has been described as a different entity as well as a part of rheumatic fever. There is a lack of validated diagnostic criteria to establish a reliable diagnosis. There are no accepted recommendations for antibiotic prophylaxis in PSRA.