Because of the wide variety of anatomic locations and patient factors, there is no one treatment that is appropriate for all desmoid tumors. The type of treatment depends on tumor characteristics and location, as well as patient characteristics and preferences. Desmoid tumors can be persistent and frustrating to manage because no one treatment modality offers a high likelihood of remission. Multiple modalities may be necessary in some patients. Although mortality is rare and is usually due to local complications, significant disability or morbidity can result from desmoid tumors, their treatment, and complications arising from treatment. The entire clinical picture and the patient's preferences must be taken into account when deciding on an appropriate treatment plan. Patients with desmoid tumors are optimally managed in a multidisciplinary setting with close collaboration between surgeon, pathologist, diagnostic radiologist, radiation oncologist, and medical oncologist. When possible, surgical resection with negative margins is the preferred modality. When surgical resection with negative margins may prove disabling, surgery can be followed by postoperative radiation, although the role and efficacy of this are controversial. In locations where surgical extirpation is difficult or unfeasible, primary radiation, hormonal therapy, or chemotherapy should be considered. Familiar adenosis polyposis (FAP)-associated mesenteric lesions, sporadic tumors present without change for months or years, or tumors present in areas where progression will not present significant additional morbidity are candidates for observation only.