The purpose of this article is to review the pertinent literature and to define the optimal treatment of patients with aggressive fibromatosis. Data indicate the likelihood of local recurrence after surgery is high, particularly if margins are positive. Moderate-dose radiotherapy alone for gross disease or after a microscopically incomplete resection yields local control rates of approximately 75% to 80%. Treatment with pharmacologic agents results in objective response rates of approximately 40% to 50%; duration of response is variable. Thus, the optimal management for aggressive fibromatosis depends on tumor location and extent. Surgery is indicated if the lesion appears to be completely resectable with acceptable function and cosmesis. Radiotherapy is indicated for patients with unresectable tumors; those with positive margins after resection should be considered for adjuvant radiotherapy depending on the location and extent of the tumor. Pharmacologic treatment should be considered for patients with progressive disease after unsuccessful local-regional therapy.