Abstract: Objective Desmoplastic small round cell tumor(DSRCT)is a rare and highly aggressive malignant neoplasm.So the diagnosis of DSRCT is difficult,and the prognosis of DSRCT is very poor.Through the study in diagnosis and therapy of DSRCT,doctor can know it very well.Methods Three patients with DSRCT were diagnosed and treated in our hospital from January 2000 to May 2010.Acourding to the domestic and foreign literature,the study investigate the diagnosis and therapy of desmoplastic small round cell tumor.Results One boy didn′t get any chemotherapy,and he died after diagnosis for 4months,the other two died from 10~14months after few cycles chemotherapy combined Ifosfamide with Etoposide and Cisplatin.Conclusion The prognosis of DSRCT is poor.Now,the main method in DSCRT is chemotherapy and surgery,but the effect is limit.So the deep investigation is necessary in integrated treatment mode of DSRCT.