We report 2 cases of desmoplastic small round cell tumor (DSRCT) involving the ovaries in young women. The first patient presented with symptoms of acute appendicitis and the second patient presented with a mass in the lower abdomen and slightly elevated CA-125 level. In both patients, the tumor was widely metastatic at presentation. The ovarian involvement was unilateral in the first patient and bilateral in the second with tumor size ranging from 9 to 11 cm. Morphology, immunohistochemistry, and molecular cytogenetics were consistent with DSRCT. Despite tumor debulking and multiple chemotherapy regimens, the first patient died at 20 months after initial diagnosis and the second is still undergoing chemotherapy at 7 months after initial presentation. To gain additional insight on DSRCT with ovarian involvement, the literature was reviewed and summarized.