A rare case of symptomatic hydrometrocolpos in a 5y old female

Late presentation of symptomatic hydrometrocolpos is uncommon. We present a 5 years old continent girl with prenatally diagnosed multicystic dysplastic left kidney and late-onset of lower abdominal pain. Investigations revealed a nonfunctioning left kidney with an ectopic ureter draining into the left hemivagina, and a vaginal duplication with an obstructed and urine-filled left hemivagina. Surgical therapy included resection of the vaginal septum and laparoscopic nephroureterectomy. Not only renal agenesis but also dysplasia or multicystic kidney may part of an OHVIRA syndrome. In girls with unilateral renal dysplasia, a duplication of the internal genitalia must always be considered.