A case is described of a 19-year-old man with poor prognosis intra-abdominal desmoplastic small round cell tumor, who achieved a durable complete remission after aggressive multimodality treatment. He was diagnosed with a large peri-rectal mass and bulky metastases to the peritoneum, omentum and liver. He achieved a partial response to six courses of chemotherapy with VAC (vincristine, adriamycin, actinomycin, cyclophos- phamide) and VIP (VP-16, ifosfamide, cisplatin). This was followed by resection of the omentum and cryotherapy to six of the liver lesions. He then achieved a complete response following high-dose busulfan 16 mg/kg and melphalan 140 mg/m2 with autologous stem cell support. Finally, pelvic radiotherapy was given to consolidate the remission. Twenty-six months following his diagnosis, and 19 months after the transplant, he remains well with no clinical or radiologic evidence of recurrent disease. This case report suggests a role for autologous stem cell transplantation as part of multimodality therapy for this unusual and aggressive malignancy.