Background: Müllerian agenesis, the second most common cause of primary amenorrhea, affects 1 in 4,000 to 10,000 women. Although the majority of these women have complete absence of the uterus, a small percentage can have a rudimentary uterus.
Case: A menopausal nulligravida with known müllerian agenesis developed a large pelvic mass and an inguinal hernia. The patient underwent an exploratory laparotomy, which revealed a large leiomyoma growing from a small rudimentary uterus, an indirect inguinal hernia, and bilateral benign serous cystadenofibroma.
Conclusion: Women with müllerian agenesis and rudimentary uteri can develop gynecologic pathology similar to women with normal genital tracts.