Ovarian cancer is the leading cause of death among women who develop gynecologic malignancies, and is the fifth leading cause of cancer death in women in the United States.1 Ninety percent of adult primary ovarian cancers are of epithelial origin and are subtyped into the following categories: serous, mucinous, endometrioid, clear cell, Brenner, and undifferentiated. Mixed epithelial ovarian carcinomas are composed of two or more of the five major cell lines of epithelial ovarian tumors. Three histological grades of epithelial tumors, ranging from well to poorly differentiated, are related to prognosis. Associated hereditary ovarian syndromes account for 5% to 10% of epithelial ovarian cancer, specifically breast-ovarian cancer syndrome and type II Lynch syndrome. Ovarian syndromes associated with epithelial ovarian cancer usually present earlier in life and with advanced staging. Current recommendations for screening women with gene mutations for these cancer types include performing transvaginal sonography and obtaining serum CA 125 levels every 6 to 12 months between the ages of 25 and 35 years. Prophylactic salpingo-oophorectomy after childbearing age also is considered.