Desmoplastic small round cell tumor (DSRCT) is a rare, highly malignant tumor with very poor prognosis. It often develops in the abdominal and pelvic peritoneum or extra-abdominal sites of adolescents and young males. Patients typically present with symptoms of abdominal sarcomatosis. The histopathological manifestation is featured with nest-distributed small round blue cells surrounded by fibrous tissue. This tumor can co-express epithelial, neural and mesenchymal markers. The molecular characteristic of DSRCT is the production of EWS-WT1 fusion protein via the translocation of chromosome t(11:22) (p13; q12). Standard management of DSRCT has not been established so far. Treatments include debulking or radical surgery, high intensity adjuvant or neoadjuvant polychemotherapy, and whole abdominal radiation. Some studies suggested that methods such as hematopoietic stem cell transplantation, targeted therapies, and postoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and intensity-modulated radiation therapy (IMRT) can improve survival. This paper reviews recent evidence-base medicine supported options for the treatment of DSRCT.