Purpose: Streptococcus pyogenes is an important cause of invasive diseases in children. We aimed to describe the clinical characteristics of invasive infections due to S. pyogenes in children in Korea. Methods: A retrospective study of children under 18 years of age with invasive infections due to S. pyogenes at Seoul National University Children's Hospital between March 1992 and December 2012, and Seoul National University Bundang Hospital between March 2003 and December 2012 was conducted. Demographic factors, clinical characteristics, laboratory findings, treatment, mortality and morbidity of all patients were reviewed. Results: A total of 30 among 36 cases identified as invasive disease due to S. pyogenes were available for review. There was a predominance for male subjects (male:female=2.75:1). The median age was 50 months (range 12 days to 15 years) and 53.3% were under 5 years of age. Skin and soft tissue infections (9/30, 30.0%), bacteremia without identified focus (4/30, 13.3%) and bone and joint infections (6/30, 20.0%) were the most frequent clinical presentations. Streptococcal toxic shock syndrome (3/30, 10.0%) pulmonary, abdomen and central nervous system infections (2/30, 6.7%) were also seen. There was a peak in number of patients in year 2012 (9/30, 30.0%). There were no cases of mortality. Erythromycin and clindamycin resistance rates were low by 3.8% and 7.5%, respectively. Conclusion: We studied the clinical presentations of invasive infections due to S. pyogenes during the past 20 years in Korean children. The findings of this study help us understand the characteristics of the disease, enhancing early recognition and prompting adequate antibiotic therapy which is important in reducing morbidity and mortality.