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Extra-abdominal desmoid tumors are rare, locally aggressive neoplasms without metastatic potential. There is no clear consensus regarding their optimal management. The disappointing results of current treatments and the ability of extra-abdominal desmoid tumors to spontaneously stabilize have increasingly drawn interest toward conservative management. The objective of this study was to evaluate a wait-and-see policy as a first-line management for extra-abdominal desmoid tumors.This two-center retrospective study involved fifty-five patients with a histologically proven extra-abdominal desmoid tumor. The primary outcome was the cumulative probability of dropping out from the wait-and-see policy. The wait-and-see policy included aggressive management of symptoms. We conducted a review of the relevant published series in which a watchful-waiting strategy was used.The cumulative probability of dropping out from the wait-and-see policy was 9.6% at the time of the last follow-up. Spontaneous arrest of tumor growth was noted for forty-seven patients (85%) over the course of the study. Half of the tumors were stabilized at one year, and a potential to increase beyond three years was a sporadic event (one case). Regrowth was found in two patients (4%).A wait-and-see policy is an effective front-line management for patients with primary or recurrent extra-abdominal desmoid tumor. These tumors tend to stabilize spontaneously, on average after one year of evolution, and the cumulative probability of the failure of a wait-and-see policy is approximately 10%.Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.