Intra-abdominal desmoplastic small round cell tumors are rare aggressive neoplasms, with a very poor prognosis, observed in young adults with a male predominance. Their etiology is unknown and the diagnosis is based on histopathology, immunohistochem-istry and cytogenetics. Histological analysis shows typically clusters of round cells separated by abundant desmoplastic stroma. These tumors exhibit a multi-marker im-munohistochemistry profile expressing the three embryonic lineages: epithelial, neural and mesenchymal. They are positive for desmin and cytokeratin and are characterized by a specific recurring translocation t (11: 22) (q12 – p13), which involves EWSR1 WT1 gene. They are usually fatal despite an aggressive multidisciplinary therapeutic approach. Hereby we report the case of a 39 year old man who presented with an in-tra-abdominal desmoplastic small round cell tumor. The diagnosis was made by radio-logical, histological and immunohistochemistry profile analyses of a CT scanguided bi-opsy. This article also includes a mini review of the literature.