Background:The desmoplastic small round cell tumor is a rare disease with poor prognosis. It affects predominantly young males with preferential involvement of the abdominal cavity. Case Report: A 33 year old male presented with right hypochondrium pain, associated with anorexia and weight loss for last 2 months. The imaging studies identified an abdominal mass, without hollow organ association, and multiple liver metastases. The anatomopathological study of the tissue obtained with the percutaneous abdominal mass biopsy confirmed the presence of desmoplastic small round and blue tumor cells. The immunohistochemical and cytogenetic study confirmed the diagnosis. It was proposed for systemic chemotherapy as primary treatment. Conclusion: We intend to emphasize that although this tumor is rare, it should be included in the differential diagnosis of intra-abdominal solid lesions. It also includes a review of the epidemiology, clinical manifestations, as well molecular and pathologic features.