Objective To investigate the clinical features, diagnosis, and treatment of patients with localized epithelioid sarcoma (ES).Methods From January 2000 to September 2006, 11 patients with ES weretreated. There were 7 males and 4 females aged 14-41 years. The patients’ agesat the initial onset were 9-41 years, averaged 27.7 years. The ES was located in the upper extremity in 7 patients，lower extremity in 3, and abdomen in 1. Among the patients, 10 had a recurrence. Tumor lt; 2cm was seen in 7 patients, 2-5cm in 1, and gt;5 cm in 3. One patient underwent an operation of local resection at another hospital. Seven patients underwent an expanding resection surgery, and the tumors with the surrounding normal tissues 3 cm above were removed. Three patients underwent a radical surgery, including extremity amputation or finger amputation. All the patients underwent routine radiotherapy and chemotherapy after operation. Results All the wounds had a healing at the firstintention without complications. All the flaps survived and the grafted bone had a fusion. Among the 11 patients followed up for 5-54 months averaged 23.2 months, 8 had a recurrence 2-20 months (average, 8.9 months) after operation, witha recurrence rate of 73%. And among the patients, 3 had a further radical surgery of extremity amputation. Four patients had a metastasis in the axillary lymphnodes 6-24 months after operation, and 1 patient had a lung metastasis 10 months after operation. They did not have a further surgical treatment. Four patients died of systemic failure 6-14 months after operation. Conclusion An early expanding resection surgery combined with postoperative chemotherapy and radiotherapy is the therapy of choice for treating ES.