Background: Isolated distal vaginal agenesis is a rare anomaly and mostly becomes symptomatic after menarche. We describe an unusual presentation of this anomaly in a prepubertal girl.

Case: An 11-year-old prepubertal girl presented with recurrent urinary tract infection, pyuria, and right-sided renal agenesis. The findings of perineal inspection, ultrasonography, and magnetic resonance imaging were consistent with a distal vaginal agenesis with pyometrocolpos. Discharging pyometrocolpos with dissection of the atretic portion and a pull-through vaginoplasty were performed. A cystoscopy showed no sign of a vesicovaginal or uterine fistula.

Summary And Conclusion: This rare presentation of distal vaginal agenesis reminds us that congenital malformations of the female genital tract should be considered in patients with congenital anomalies of the urinary system and/or recurrent urinary tract infection, even during the prepubertal period.

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http://dx.doi.org/10.1016/j.jpag.2016.08.007DOI Listing

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