Background: The outcomes for the patients (pts) with adult RMS have remained poor, with approximately 30% at 5-year survival. The purpose of the current study is to compare the clinical outcomes of adult RMS to childhood RMS by local or metastatic disease, to examine the impact of local therapy, and the optimal timing of local therapy in metastatic disease. Methods: We collected the data of the pts with RMS between 1981 and 2010 at our institution. The chart review was performed for all identified pts to obtain the following information; age, stage, group, disease site, histopathology, chemotherapy regimen, tumor response, and radiotherapy schedule. We defined adult as the pts aged greater than or equal to 21 years. The induction phase was defined as in the first 6cycles of VAC and the maintenance phase was defined as after the first 6 cycles of VAC. The progression free survival (PFS) and overall survival (OS) were evaluated using the Kaplan-Meier method and the Cox hazard model. Results: We identified 98 pts. Of 36 pts were adult (median age, 29; range, 21 to 72) and of 62 pts were childhood (median age, 11; range, 0.6 to 20). The median PFS of localized and metastatic disease for childhood and adult RMS were as follows, localized disease, 166.9 vs. 22.4 months (p = 0.005) and metastatic disease, 13.3 vs. 13.3 months (p = 0.949), respectively. Multivariate regression analysis demonstrated that age (< 21 vs. ≥ 21) was negatively correlated with PFS (p = 0.044) and the tumor response (PD / SD vs. PR / CR) was positively correlated with PFS (p = 0.005) in the localized disease. In 20 pts with metastatic disease, only the local therapy (surgery and / or radiotherapy) significantly improved the PFS and OS while age did not relate to survival. We further examined the optimal timing of local therapy in 53 patients who received local therapy during the induction phase or the maintenance phase. The differences of PFS and OS were not statistically significant among the patients. Conclusions: The age was a negative prognostic factor of PFS in RMS with localized disease. For metastatic disease, local therapy may be effective on survival but the timing of local therapy can be varied depending on pts.