PURPOSE: With the use of combined-modality therapy, the survival of the patients with the rhabdomyosarcoma (RMS) has been improved during past 30 years. The reports on the therapeutic outcome of RMS in Korea during past 10 years, however, have been scarce. The authors reviewed the clinical characteristics and treatment outcome of pediatric RMS patients diagnosed in our institution during recent 11 years' period and compared this data with previous reports. METHODS: From January 1990 to December 2000, 29 patients were diagnosed with RMS by histopathology. Retrospective analysis of the medical records of these patients was performed. RESULTS: A male to female ratio was 1.4:1. Age at diagnosis ranged from 8 months to 19 years 5 months and the median age was 8 years 5 months. Head and neck region that included orbit and parameningeal areas was the most common primary site (44.8%) and other common sites were genitourinary tract, extremities and perianal areas. The two major pathologic types of RMS were embryonal (72.4%) and alveolar (17.2%). The patient distribution of Intergroup Rhabdomyosarcoma Study (IRS) clinical group was as follows: Group I 13.8%, Group II 24.1%, Group III 27.6% and Group IV 34.5%. Of the 29 patients, 4 patients were lost to follow up or received no treatment. Among 25 patients treated and followed, 3-year overall survival rate of these patients was 84% and 3-year event-free survival (EFS) was 60%. Three-year EFS of Group I was 100%, Group II 71%, Group III 83% and Group IV 22%. CONCLUSION: The clinical characteristics of RMS in our institution were similar to those reported by IRS. But there were higher proportion of Group IV patients than IRS suggesting the delayed diagnosis of RMS in Korean patients. The treatment outcome was comparable to that of current IRS trials. This results suggest that the treatment outcome of RMS patients in Korea has improved largely owing to the adoption of current IRS protocols.