Résumé
Peritoneal desmoplastic small round cell tumor is a very rare malignant neoplasm and has specific clinical features; It is predominant in children and young males and has a well-demarcated large intra-abdominal tumor, which has not been associated with a primary visceral organ, with diffusely scattered multiple small tumors and rarely involves ovaries. It is a very aggressive and fast growing tumor along the peritoneal surfaces of the abdomen and pelvis. It has a typical histologic features and a specific immunohistochemical staining pattern. There is no definite treatment. It responses to surgery and chemotherapy at early period of therapy but relapses soon and rapidly progresses and then causes the death. We have experienced a peritoneal desmoplastic small round cell tumor which involved both ovaries, so we report this case with a brief review of literature.