Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare anomaly in women and is characterized by congenital aplasia of the uterus and vagina, with normal development of secondary sexual characteristics and a normal karyotype. We report a case of a 38-year-old women with MRKH syndrome that had experienced urethral sex for many years. She presented with urinary incontinence and dyspareunia. The patient's secondary sexual characteristics were normal, and examination revealed a widely open incompetent megalourethra and an absent vagina. Laboratory studies confirmed a 46, XX karyotype. Imaging included ultrasonography and magnetic resonance imaging, which indicated bilateral normal ovaries and a rudimental bicornuate uterus. After confirming the diagnosis of MRKH, the patient underwent urethroplasty by urethral plication, antiincontinence surgery by autologous fascial sling of the bladder neck, and the creation of a neo-vagina using a urethral flap. After 3 months, voiding cystourethrography and uroflowmetry confirmed normal voiding. There were no postoperative symptoms of urinary incontinence, and the patient was completely satisfied.
Keywords: Plastic surgery; Urethra; Urinary incontinence.