Intra-abdominal desmoplastic small round cell tumor is an extremely rare and aggressive neoplasm that predominantly occurs in young adult men; it has an uncertain histogenesis, and predominant or exclusive intra-abdominal localisation without visceral origin involvement. This tumor is characterized by its distinct morphology and its multiphenotypic differentiation. We report a case of an intra-abdominal desmoplastic round cell tumor in a 24 years old woman. In the light of this case, the clinical morphological immunohistochemical and molecular of this rare desease are revewed.