BACKGROUND Considering diversity and rarity of non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS), multi-institutional study on NRSTS is indispensable. As a pre-step for devising multi-institutional study format, we investigated clinical characteristics of NRSTS patients in a single center. PATIENTS & METHODS Retrospective analysis was made on medical records of 23 patients who were hospitalized with the diagnoses of NRSTS to National Cancer Center, Korea from April 2003 to May 2009. Demography, pathologic diagnoses, treatment modality, and outcome were examined. RESULTS Mean age of 23 patients was 13.0 (range 0.1-32.0) years. Male to female ratio was 1:1.6. Synovial sarcoma (n= 5) was most commonly diagnosed followed by alveolar soft part sarcoma (n= 2), malignant fibrous histiocytoma (n= 2), malignant peripheral nerve sheath tumor (n= 2), clear cell sarcoma (n= 2), mesenchymal chondrosarcoma (n= 2), desmoplastic small round cell tumor (n= 2), sarcoma, not otherwise specified (n= 2), fibrosarcoma (n= 1), hemangiopericytoma (n= 1), dermatofibrosarcoma protuberans (n= 1). Twenty patients had primary diseases and 3 recurrent ones. Nine patients (39.1%) had metastatic diseases at diagnosis. Of 13 metastatic sites, lung (n= 6) was most commonly involved followed by brain (n= 4), and bone (n= 3). Of 22 patients who were given treatment, 21 (95%) underwent chemotherapy, 20 (91%) surgery, and 11 (50%) radiotherapy. Chemotherapeutic regimens utilized were VCD/IE (15.5%), ICE or VICE (15.5%), CCG7921B (4.4%), CCG4941 (4.4%), VIA (4.4%), VI (4.4%), and various other regimens (55.8%). Fifteen patients (68.2%) had been in CR, 1 (4.5%) in PR, 3 (13.6%) in SD at last follow-up, and 3 (13.6%) died of disease progression. Five-year OS and EFS were 83.0% and 38.9%, respectively with a median follow-up of 33.6 (range 2.4-60) months. Of 8 events, 4 relapses, 3 PD, 1 second malignancy were reported. CONCLUSIONS Clinical characteristics and outcome of NRSTS in our study population were not quite different to those described in the literatures. However, in view of very small number of individual disease within NRSTS, large patient pools obtained by multi-institutional study are urgently needed.