Abstract: Objective To investigate the clinical characteristics and treatment methods on peripheral primitive neuroectodermal tumors(pPNET) patients. Methods We analyzed retrospectively the clinicopathological characteristics and treatment methods on 10 pPNET patients treated in the First Affiliated Hospital of Chongqing Medical University from November 2005 to April 2014 and calculated the survival rate by direct method. Results Ten cases were mainly male teenagers. Tumor cells strongly expressed CD99 and NSE. Six patients were treated with surgical resection, among which five patients received postoperative radiotherapy and chemotherapy, two patients received radiotherapy and chemotherapy, one patient received chemotherapy and HIFU and one patient was treated with Chinese medicine. The 1-, 2- and 5-year survival rates of the patients were 80.0%(8/10), 71.4%(5/7) and 40.0%(2/5), respectively. Conclusion The clinical characteristics of pPNET patients are lack of specificity. The diagnosis of pPNET mainly depends on histology and immunohistochemistry. The multimodality therapy of pPNET includes surgery, radiotherapy and chemotherapy.