Mayer-Rokitansky-Kuster-Hauser syndrome associated with endodermal sinus tumor of the ovary.

Both EST and Mayer-Rokitansky-Kuster-Hauser syndrome are uncommon disorders. This report illustrates a case of these two rare conditions occurring in the same patient. Patients with Mayer-Rokitansky-Kuster-Hauser syndrome, although not appearing to be at increased risk for germ cell tumors, unlike testicular feminization patients, are not immune to ovarian cancer. Therefore, individuals with Mayer-Rokitansky-Kuster-Hauser syndrome should still be followed gynecologically because they are at least at the normal risk of developing ovarian as well as other gynecological neoplasms.