Small cell carcinoma of the ovary has been identified as a specific histopathologic entity. The first II cases were reported by Dickersin et a1, 1982, Since then only 80 cases were reported in literature. This rare and highly aggresive melignancy primarily affeets ehildren and young women(10-40) and grows rapidly, with hypercalcemia occuring frequently. Abscence of effective treatment results in early mortality. Recently we have experienced one case of primary sma11 cell carcinoma of the ovary, which is presented with a brief review of the literature.