Abstract
Desmoplastic small round cell tumour corresponds to a rare and aggressive neoplasm, individualised in 1989. The clinical characteristic includes a predilection for young adult men and predominant intra-abdominal peritoneal localisation. This condition causes etiopatogenic, diagnostic, therapeutic and prognostic problems. Indeed, the etiopathogenisis is still unknown. Diagnosis is asserted only by immunohistochimic and cytogenic study. Its treatment is not well codified. The prognosis is poor because this tumour is relatively unresponsive to association of surgery, chemotherapy and radiation therapy. The objective of this work is to report a personal observation of a desmoplastic small round cell tumour, and to proceed to a review of the literature to clarify the epidemiological, clinical, para clinical and therapeutic aspects of this rare tumour.