Desmoid tumor (aggressive fibromatosis), as a member of a group of borderline neoplasms, is a rare tumor of fibroblastic origin that remains difficult to treat. Treatments with surgery, radiotherapy and different medical protocols including interferon (IFN)-alpha, hormonal agents such as tamoxifen (anti-estrogen) as well as non-steroidal anti-inflammatory drugs and low-dose antineoplastic agents have been reported. In this report we describe a new patient with multifocal aggressive fibromatosis who was successfully treated with low-dose chemotherapy consisting of methotrexate and vinblastine.