Objectives/Hypothesis Low‐grade myofibroblastic sarcoma (LGMS) is a rare entity that is described as having a predilection for occurring in the head and neck region. Here we analyze its demographics, clinic‐pathologic, and survival characteristics. Study Design Retrospective database analysis. Methods A cohort from the Surveillance, Epidemiology, and End Results Program database of cases with LGMS between 2001 and 2012. Results There were 49 cases with a 5‐year overall survival of 71.6% and disease‐ specific survival of 76.3%. The majority of cases were in patients <60 years old, female, and white ethnicity. The most common sites were the extremities in 40.8% of cases followed by the head and neck region with 26.5% of cases. Multivariate analysis showed that only older age was significantly associated with worse survival (P < .05). Conclusions LGMS is uncommon in the United States and occurs most commonly in the extremities followed by the head and neck region, despite an existing characterization of a predilection for the head and neck region. Treatment most commonly involves surgery, but the optimal surgical extent and/or radiotherapy needs to be further investigated. Level of Evidence 2c Laryngoscope, 127:116–121, 2017