Obstructed hemivagina with ipsilateral renal anomaly with ureteric remnant in a prepubertal female

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn–Werner–Wunderlich syndrome, is a rare Müllerian duct anomaly that is characterized by a triad of uterus didelphys, unilateral obstructed vagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with abdominal pain or a pelvic mass which worsens over some time. Herein, presented is a case of a 12-year-old nulligravid with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and nonfunctional urinary bladder. The patient underwent diagnostic hysteroscopy, cystoscopy, and full resection of the longitudinal vaginal septum as well as drainage of hematocolpos, which are the main treatment of the patient with OHVIRA.