A follow-up study of 53 patients with extra-abdominal desmoid tumor is presented. Fine-needle aspiration biopsy seemed to be valuable in the differentiation between a desmoid tumor and a truly malignant soft tissue tumor. The angiographic pictures did not differ from those of other soft tissue tumors, but valuable topographic information was obtained from the angiography in the preoperative planning. The overall local recurrence rate was 17/44, and 15 of the recurrences occurred within the first 2 years. Complete removal of the affected muscle or muscle group is recommended. Local control can be achieved by non-ablative surgery even after multiple recurrences. Only one out of 53 patients died of the tumor disease. Radiotherapy can be attempted in extensive tumors, and amputation should be reserved for tumors which have failed to respond to this treatment.