Aggressive fibromatosis is a rare fibroproliferative disorder with a variable biologic potential that is locally morbid but does not metastasize. Eighteen patients with extraabdominal fibromatosis were treated with a multidisciplinary approach over a 27-year period. Our observations, coupled with a review of the literature, suggest that conservative surgery with the goal of a wide margin coupled with adjuvant therapies may result in adequate control of disease from infancy to adolescence. Amputation should be reserved for cases in which the disease or its treatment have resulted in a nonfunctional or chronically painful extremity. Radiation should be used as a last resort in the skeletally immature because of the risk of growth disturbance, contracture, and secondary malignancy. Chemotherapy may have a role in children with inoperable disease, in those who have gross residual tumor after an intralesional procedure, for disease progression or recurrence, and neoadjuvant therapy should be investigated as a means to achieve a wide margin in some cases.