Clear cell adenocarcinoma of ovary is a rare tumor and represents less than 5% of all malignant ovarian tumors. It is an entity distinct from epithelial carcinoma of the ovary and may result from malignant transformation of endometriosis, usually unilateral and highly aggressive. Surgery and chemotherapy are therapeutic weapons even if this tumor has a poor prognosis. We are reporting a case of clear cell adenocarcinoma of ovary, on a young pauciparous patient whose initial therapeutic treatment was effective.

Keywords : Clear cell adenocarcinoma, Endometriosis, Surgery, Prognosis