Extraskeletal myxoid chondrosarcomas (EMC) are rare soft tissue sarcomas of uncertain lineage that have an indolent course yet high risk of relapse. Due to a paucity of data, the role of radiation therapy (RT) in their management is poorly defined. Therefore, given our comparatively large experience managing patients with EMC, we sought to evaluate outcomes in this rare tumor type for patients with localized disease. We reviewed the records of 41 patients with primary ECM treated at our institution from 1990 to 2016. The Kaplan-Meier method was used to estimate rates of overall survival (OS), disease-specific survival (DSS), local control (LC), and distant metastatic free survival (DMFS). Median follow-up time was 94 months (range, 8-316). The median patient age was 53 years (range, 20-84) and predominantly male (n=34, 83%). The most common tumor location was the lower extremities (n=22, 54%; UE n=10, 24%; trunk n=8, 20%; H&N n=1, 2%) with a median tumor size of 7.5 cm (range, 2-38), and the majority were >5cm (n=32, 78%). Most patients (n=33, 80%) received combined modality therapy (CMT) with surgery and RT, whereas only 8 (20%) underwent surgery alone. Median OS was 89 months (range, 8-316 months) with a 5- and 10-year OS rate of 88% and 66%, respectively. There were 5 deaths (12%) that were attributable to disease with a median survival of 74 months (range, 65-122 months) which resulted in a 5- and 10-year DSS of 100% and 85%, respectively. There were no patient or treatment-related factors associated with DSS, but distant relapse did negatively impact DSS (10-yr 62% vs. 100% if no DM, P=0.002) without influencing OS (10-yr 62% vs. 68% if no DM, P=0.52). The 5- and 10-year LC rates were 94% and 90%, respectively. There were 5 patients (12%) with local relapse at a median time of 75 months (range 13-176 months). On univariate analysis, the only significant factor associated with poorer LC was the use of surgery alone (10-year LC 63% vs. 100% for CMT, P=0.004), which remained the only factor also significant on multivariable analysis (P=0.02, HR 12.7, 95% CI 1.4 -115.3). Thirteen patients (32%) developed DM at a median time of 28 months (range, 3-154 months). The 5- and 10-year DMFS rates were 73% and 69%, respectively. Two factors were associated with poorer DMFS on univariate analysis including tumor size >5cm (5-yr 59% vs. 100% ≤5cm, P=0.02) and local relapse (5-yr 60% vs 74% no local relapse, P=0.05). Interestingly, local recurrence was the only factor associated with poorer DMFS on multivariate analysis (P=0.04, HR 3.9, 95% CI 1.1-14.7). Following disease relapse, the median DSS was 59 months (range, 25-241 months) with a 5- and 10-year DSS after relapse of 75% and 50%, respectively. For patients with EMC, CMT resulted in improved LC, which was important given that local relapse was associated with poorer DMFS. Achieving upfront LC with CMT may reduce the risk of DM. Despite the high risk for metastatic relapse, patients survive for long periods of time with distant disease.