A rare form of Mayer-Rokitansky-Küster-Hauser syndrome: Case report and review of literature.

Introduction: Primary amenorrhea is failure to reach menarche. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea, after gonadal dysgenesis. Herein, we report a rare case of an unusual subtype of MRKH syndrome.

Case Report: This report describes the case of a 28-year-old woman with primary amenorrhea and coital difficulties. On examination, she had normal secondary sexual characteristics but a blind-ending vagina. Her hormonal profile was within normal limits; ultrasound examination was inconclusive due to technical difficulties. Subsequently, she underwent pelvic magnetic resonance imaging, which revealed bilateral rudimentary uterine horns in the presence of normal ovaries and normal length of a blind-ending narrow vagina.

Conclusion: MRKH syndrome with bilateral rudimentary uterine horns and normal-length vagina is an unusual form of Müllerian agenesis.