Sclerosing Epithelioid Fibrosarcoma (SEF) is a rare form of soft tissue sarcoma. It is characterized by a slow evolution, with local recurrences and late metastases that are mainly pulmonary and pleural in about 50% of cases. The treatment is based on the surgery which must be as wide as possible. The efficacy of adjuvant therapy in the control of SEF is not yet demonstrated. Chemotherapy is used in recurrences, some have also proposed radiotherapy. Long-term follow-up of patients with SEF is therefore essential.