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Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm of uncertain histogenesis that preferentially involves the abdominal and pelvic cavities. DSRCT mainly develops in adolescent and young adults with a strong male predominance; the male to female ratio is 4:1. Ovarian location is exceptional. DSRCT generally develops in the abdomen and have a tendency towards peritoneal spread, with subsequent metastasis to distant lymph nodes, liver and lungs. It is a poorly understood malignancy with a very characteristic morphology, immunophenotype, cytogenetic features, and poor prognosis. This tumor can co-express epithelial, neuronal, and mesenchymal markers. Despite intensive therapy, including surgery, radiotherapy and chemotherapy, and immunotherapy; the 5-year survival is less than 15%.