The treatment of extraabdominal desmoid tumors remains controversial. Although a radical resection is associated with lower recurrence rates, the treatment of extensive, limb-threatening, or multifocal desmoids may require a more conservative excision with postoperative radiation and selective hormonal therapy. This approach would minimize functional deficits and improve cosmesis. If conservative resection with adjuvant radiotherapy and endocrine manipulation has failed and the tumor is compromising vital functions, then more radical resections may be justified.