Objective. The aim of this study is to investigate the association of different subgroups of juvenile rheumatoid arthritis (JRA) with human leukocyte antigen (HLA) class II DR alleles. Methods. One hundred and nineteen Korean juvenile rheumatoid arthritis patients were classified as HLA-DRB1 allele. To assess the frequency, phenotype frequencies of all JRA cases and each subtypes were compared to those of 485 adult controls. Results. HLA-DRB1*01 was associated with increased risk of JRA. Furthermore, DRB1*01 was associated with polyarticular JRA and pauciarticular JRA. The frequencies of DRB1*14 and DRB1*15 were higher in systemic JRA patients than the controls. Conclusion. The data of this study on Korean children with JRA suggests that HLA-DRB1*01 was associated with the susceptibility of JRA. The study should be extended to include larger numbers of patients.