Desmoplastic Small Round Cell Tumor (DSRCT) has a very poor prognosis. This report illustrates novel chemotherapy and local control interventions in a 5-year old patient. The patient was treated in the outpatient setting, achieved remission, with excellent quality of life. The patient presented with massive ascites and >1000 abdominal tumors. Neoadjuvant chemotherapy included vincristine (1.5 mg/m(2)), ifosfamide (3 g/m(2)/day x 3), dexrazoxane/doxorubicin (750/75 mg/m(2)), and etoposide (150 mg/m(2)). Continuous hyperthermic peritoneal perfusion (CHPP) with cisplatin (100 mg/m(2)) was given after extensive cytoreductive surgery. This was followed by irinotecan (10 mg/m(2)/day x 5 x 2 weeks) + temozolomide monthly x 2, then abdominal radiation 30 Gy with simultaneous temozolomide (100 mg/m(2)/day x 5). A total of 12 cycles of irinotecan and temozolamide were given. Except for initial chemotherapy, subsequent courses were in the outpatient setting. Focal retroperitoneal relapse at 18 months was treated with IMRT with bevacizumab (5 mg/kg) and 2 perihepatic metastases with radio frequency ablation/cryoablation followed by chronic outpatient maintenance chemotherapy (valproic acid, cyclophosphamide, and rapamycin). Almost 2 years from diagnosis, the patient maintained an excellent quality of life. This is a novel approach to the treatment of children with massive abdomino-pelvic DSRCT.