At the end of the 20th century, after an apparent decline, acute rheum
atic fever (ARF) now constitutes a great challenge for developed and d
eveloping countries. It is caused by a group A beta-hemolytic Streptoc
occus upper airways Infection, but the exact pathogenetic mechanisms a
re not yet clear. The role of the immune system in the pathogenesis of
ARF is understood better than genetic host factors. ARF can mimic man
y other diseases, and the diagnosis is based on clinical criteria. It
is still overdiagnosed and underdiagnosed in different settings. Penic
illin has greatly contributed to the reduction in the incidence and re
currence of this disease. Current schemes of prophylaxis, however, pre
sent many problems, and failures are common. Future efforts to reduce
the burden of this disease should induce public health measures and va
ccine strategies.