Previous reports even large studies discussing the prognosis of desmoids have included tumors from intra- and extra-abdominal sites as well as incomplete resection. The purpose of this study was to explore prognostic factors associated with the recurrence free survival (RFS) rate in surgically treated extra-abdominal and abdominal wall desmoids. A total of 198 consecutive desmoid patients were treated with surgery over a 20-year period at a single institution. Of these, 151 patients with extra-abdominal and abdominal wall tumors were retrospectively reviewed. One hundred thirteen patients were referred for the primary tumor and the other 38 for recurrent disease initially treated elsewhere. All patients underwent a macroscopically complete resection. The median follow-up interval was 102 months. Thirty-one patients (20.5%) had a local recurrence (LR). No patients died of the disease. The 5- and 10-year RFS was 79.7% and 78.5%, respectively. Admission status, gender, tumor size, margin status, location, and number, were predictors of LR in univariate analysis. Tumor size and margin status were independent prognostic factors in multivariate analysis. Positive margins were predictive of recurrence of primary disease, and also showed a trend for recurrent disease, which was not statistically significant. The selective use of adjuvant radiation did not show significant benefit over local control. Regardless of primary or recurrent disease, microscopically negative margins should always be the goal for extra-abdominal desmoids surgery, if no cosmetic defects or function demolition is encountered. Extra-abdominal desmoids deserve more attention and should be treated more aggressively, especially when leaving positive margins.