Objective: To report on a patient with Müllerian agenesis (MA) and inguinal ovaries and to review the pertinent literature.
Design: Case report and literature review.
Setting: Clinical.
Patient(s): A 12-year-old girl.
Intervention(s): Magnetic resonance imaging, review of prior ovarian biopsies, karyotyping.
Main outcome measure(s): Accuracy of diagnosis and preservation of ovarian function.
Result(s): A 12-year-old girl with MA and bilateral inguinal ovaries is reported. An exhaustive review of the English literature on this rare association revealed only seven other well-documented such cases. Management of this condition involves reduction of the ovaries into the pelvis.
Conclusion(s): The association of MA and bilateral inguinal ovaries is rare and possibly underreported. Ideal management consists of surgical reduction of the gonads into the pelvic cavity as soon as they are discovered, to decrease the risk of ovarian torsion and loss of ovarian function.