Abstract: Objective To analyze the clinical features,treatment,and prognosis of proximal-type epithelioid sarcoma(PES).Methods The clinical data of 7 cases with PES in the First Hospital of Peking University from January 2000 to May 2013 were collected and analyzed,including clinical features,pathology,diagnosis,treatment and prognosis.Results All 7 patients with clinical manifestations of slowly growing painless subcutaneous nodules in the head,neck and trunk received extensive resection surgery.The majority(5/7)of patients received radiotherapy postoperatively,and the survival rate of 1-year,3-year and 5-year in our whole group was 100%,80% and 60 %.Conclusion The prognosis of proximal-type epithelioid sarcoma is poor because of its high risk of local recurrence,node metastasis,and distant metastasis.PES is rare clinically and the pathological diagnosis is required.The early extensive resection surgery is still the main treatment of PES.