Four cases of clear cell adenocarcinoma of the genital tract in young women are presented. All patients were treated with radiation therapy. Two of the patients subsequently developed chest metastases. Three patients have died of the disease, and the other is without evidence of disease. Two patients were treated with chemotherapy, and a positive response to the chemotherapy was observed. Because this is an aggressive disease, one should consider the use of chemotherapeutic agents as a part of the primary therapy.