Since 1985 there have been worldwide reports of increases in severe invasive Group A streptococcal (IGAS) infections. We reviewed the charts of all children with IGAS infections (defined as isolation of Group A streptococcus from a normally sterile site) presenting to our institution over a 7-year period (January, 1985, to December, 1991) and the literature. Streptococcal toxic shock syndrome required hypotension and multisystem organ involvement. Twenty-four patients (mean age, 4.96 +/- 4.4 years) were identified with IGAS infection. One patient (presenting in 1989) met the criteria for probable streptococcal toxic shock syndrome and none died. Eight of 19 Group A streptococcal isolates tested were streptococcal pyrogenic exotoxin (SPE) A producers, most (90%) had the speC gene and all had the speB gene and produced the toxin. No M or T type predominated. The low rates of streptococcal toxic shock syndrome and fatalities among children with IGAS infection are consistent with other pediatric but not with adult series. The apparent differences in outcome of IGAS between children and adults were not explained by the virulence factors we examined and may warrant further investigation.