Abstract: Objective To study the diagnosis,treatment and prognosis of embryonal rhabdomysarcoma（ERMS）. Methods Clinical data about one ERMS patient admitted to our hospital in March 2011 were reported.Domestic and foreign literature on misdiagnosis of ERMS in female reproductive system was collected and information on the treatment modalities for ERMS patients with a tumor-free survival time for over 30 months was analyzed. Results Of the 4 reported misdiagnosed cases,1 was finally diagnosed as ERMS by autopsy,the other 3 misdiagnosed cases were treated with local surgery and follow-up treatment with a poor outcome.Thirty-nine cases with a tumor-free survived time for over 30 months were reported and early stage ERMS accounted for 87.88%.The prognosis of these patients was rather good after surgery-based active treatment. Conclusion The incidence of ERMS is low,especially in the female urogenital system.The diagnosis of ERMS mainly depends on its clinical symptoms,pathological and immunohistochemical examination.It is mainly treated with surgery and its prognosis is poor.