Abstract: Objective: To study the clinical characteristics, treatment and prognosis of the ex-tracranial primitive neuroectodermal tumors (PNET). Methods: A total of 19 patients diagnosed with extracranial PNET underwent treatment in our hospital from January 1998 to January 2006 were partici-pated in this retrospective study. The survival rate was calculated by Kaplan- Meier. Results: The over-all survival time was 0.2-4.5 years and the median survival time was 1.4 year, among these, 2-year survival rate was 39.0%. Seven out of 10 patients who underwent radiotherapy recrudesced after differ-ent period, comparing with 6 patients without radiotherapy and 3 of them had recurrence, indicated ra-diotherapy couldn't reduce the local failures (χ2 =0.64, P=0.42). The 2-year survival rate of 13 patients who received conventional chemotherapy under tolerated dose was 42.2% in contrast with 6 patients who didn't receive chemotherapy 33.3%, implicated that conventional chemotherapy couldn't prolongthe survival (P=0.45). Recovery of serum lactate dehydrogenase after treatment showed as a prognostic predicator statistically significant correlated with disease progression. Conclusion: The conventionalchemotherapy and radiotherapy can't achieve effective therapeutic outcome of extracranial PNET and followed by poor prognosis. Higher dose chemotherapy and extensive surgery in treatment of extracra-nial PNET need to be further investigated.