Methods: The data of 25 cases of head and neck ERMS in our hospital from 2005.May to 2011.Feb werereviewed, and analyzed, and literature review is presented. Results: There are 19 young patients (<2lyear) and 6adult patients (>2lyears). 8％ patients (2/25) are lost to follow up. The median survival time is 42 months. Themean age is 15.8 years. There are 5 patients with local diseases (stage I) and 10 patients (40%) with directinvasion of lymph nodes. Only one patient has metastatic disease. 6 patients experienced relapsed diseases. Allpatients received resection or biopsy. 24 patients received chemotherapy. 13 patients (52%) achieved completeremission (CR), so did most stageⅠ or Ⅱ patients (10/13). staging is closely connected with prognosis (p=0.015,log -rank test). Conclusion: Early diagnosis and treatment is very important. Combined -modality therapycomprising chemotherapy, radiotherapy (RT) and surgery is the primary treatment for all patients so far. It isimportant that each individual have a treatment plan that addresses his/her individual needs. Target therapy willlikely play a more significant role in the future as a tangible treatment option.