Background: Rhabdomyosarcoma is a rare malignant soft tissue sarcoma and the most common sarcoma of childhood. The purpose of this study was to investigate the distribution of age and sex of patients, pathological subtypes and to determine the role of staging and classification of RMS on treatment outcome. Materials and Methods: This retrospective study included patients who diagnosed with RMS in Cancer Institute between 2006 and 2013. We used the Kaplan Meier and life table methods to estimate survival rate. STATA statistical software ver. 11.2 was used for statistical analyses. Results: Thirty patients with a mean age of 15.1 years (±SD =18.1) were evaluated.66.6 Percent were males. Median follow-up for survivors was 57.3 months (±SD=32.4). Survival rates of patients were as follows: 6 months (94%), 1 year (87%), 3 years (69%), and 5 years (50%). The pathology review classified of tumors as botryoid (6.7%), spindle cell (6.7%), embryonal (40%), alveolar (33.3%) and undifferentiated (13.3%). 3 years survival for patients with international classification III (alveolar and undifferentiated) was 70%, International classification II (embryonal) was 67%. Due to small sample size, we can’t report 3 years survival for international classification I (botryoid and spindle cell). Conclusion: Our results support early age of onset (>50% of RMS cases are diagnosed before age 10 years). Age of diagnosis often gives key facts about clinical behavior and has a noticeable effect on 5 year survival it is a prognostic factor in RMS. Disease extent as well as age and histology affect survival.