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 lower abdominal pain. Investigations revealed nonfunctioning an ectopic ureter draining into the hemivagina, vaginal duplication obstructed urine-filled hemivagina. Surgical therapy included resection septum laparoscopic nephroureterectomy. Not only renal agenesis but also dysplasia or may part OHVIRA syndrome. In girls unilateral dysplasia, internal genitalia must always be considered.