A case of adenosquamous carcinoma arising in the background of disseminated pelvic endometriosis presented as unilateral hydronephrosis and a polypoid intraluminal ureteral mass. This is the first case of a malignancy arising in endometriosis presenting as an obstructive ureteral mass. The patient had a history of total hysterectomy and bilateral salpingo-oophorectomy 5 years earlier because of an endometriotic cyst, and had since been under unopposed estrogen replacement therapy. An analysis of the case and related literature is presented. Possible pathogenic mechanisms are discussed.