Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is an unusual and special type of Müllerian duct anomaly. Patients usually present in adolescence, soon after menarche, with pain and pelvic mass and rarely with infertility in adulthood. Majority of the cases of OHVIRA have been reported in association with uterus didelphys and the presentation of uterus didelphys with OHVIRA is known as Herlyn-Werner-Wunderlich syndrome. A complete septate uterus with OHVIRA is exceedingly unusual. Less than 30 cases of complete septate uterus with OHVIRA have been reported to date, to the best of our knowledge. We present a rare case of incidentally detected complete septate uterus with OHVIRA in a young woman who presented with acute pain in the right iliac fossa owing to acute appendicitis.