Although musculoaponeurotic fibromatosis (MAF) is a well-recognized entity, it still provides a histologic diagnostic enigma, particularly in its distinction from a well-differentiated fibrosarcoma. The lesion is histologically identical to a desmoid tumor. The lesion appears as a firm swelling in a limb or limb girdle, with a rare incidence of pain. Although it is more common in the second to fourth decades (premenopausal women predominantly), it does occur in children. The sex incidence in children is equal. A multicentric pattern of behavior may be evident. The mainstay of treatment has been surgical excision of varying extent. Local excision, even if radical, may be followed by up to a 65% recurrence rate. In this review, 40% of the cases had more than one recurrence. Recent evidence suggests radiotherapy, which has been reserved for inoperable lesions, may be of value as an adjunct to primary treatment. Limited local excision with adjunctive radiotherapy may decrease recurrence rate, minimize functional deficit, and improve cosmesis. Radical local resection (compartmental or amputation) may be avoidable. In children, amputation may not result in cure because of the multicentric behavior pattern and radiotherapy may lead to growth retardation. A shorter functional upper or lower limb is preferable to an amputation stump or a limb disfigured from radical excision.