A retrospective analysis of the clinico-pathologic aspects of 22 cases of clear cell ovarian carcinoma with a literature survey is the subject of this report. The rarity of these neoplasms below the age of 40 is reaffirmed. Tumor-related hypercalcemia was observed in two patients and postoperative thromboembolic complications were encountered in three others. Electron microscopic examination revealed abundant cytoplasmic glycogen content in two cases. Ten patients in this group and 26% in reported series had coexistent endometriosis. Association with endometrial carcinoma was observed in two patients and was reported in 14% of the cases. No patient of ours with Stage III or IV disease survived 5 years or longer and only 8% have reportedly survived in collective series. There was suggestive evidence of radiation tumor response in two patients with Stage IIC disease who had received 5,000 rads pelvic radiotherapy. Objective partial responses were also observed with adriamycin-, cytoxan-, and cis-platinum-containing combinations. A management plan is outlined.