The authors report a case-history of lymphangiosarcoma or Stewart-Treves syndrome which occurred in a patient with posttraumatic lymphedema of the arm. He presented two recurrences after surgery which led to the decision of amputation. He is now disease free nine years after this treatment. Initially defined as "lymphangiosarcoma arising in chronic lymphedematous extremities after mastectomy for breast cancer", Stewart-Treves syndrome complicates rarely traumatic, postsurgical, postradiation, idiopathic, congenital or filarial lymphedema. Clinical diagnosis is based on nodular, purple and frequently multiple skin lesions on chronic lymphedema of the limb. A proliferating malignant endothelium with affinity for anti-factor-VIII is found on histological slides. The tumor aggressivity explains the short survival if no treatment is given. Stewart-Treves syndrome prognosis is poor, with the occurrence of multiple local recurrences and pulmonary metastasis. On account of its rarity, there is no therapeutic consensus. In localized stage, radical surgery is usually performed. Conservative treatment with complete tumour removal and postoperative radiotherapy has not been yet evaluated. In metastatic or locally advanced tumours, it is necessary to study the benefit of cytotoxic drugs like anthacyclins and ifosfamide known to be effective on soft tissue sarcomas.