Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex Mullerian anomaly with an incidence of 1:20,000. The commonest presentation is pain in the abdomen and progressive dysmenorrhea. Other rare symptoms could include vaginal discharge, fever, infertility, or acute abdomen. Case description: A 15-year girl presented with acute retention of urine which required catheterization. She had attained menarche 8 months back. On evaluation, the ultrasonography report revealed the presence of uterine didelphys with right side pelvic collection and absent kidney on the same side of the collection. On magnetic resonance imaging (MRI), a right obstructed hemivagina was clearly seen along with other findings suggesting the OHVIRA syndrome. Laparoscopically, a bulge was seen just below the right uterine horn which caused the retention of urine. Septal resection was performed vaginally, following which her symptoms subsided. Clinical significance: Suspect OHVIRA syndrome in adolescent girls when there is a renal anomaly with Mullerian defect. The patient can also present with acute retention of urine because of hematocolpos. Early detection and treatment will help to prevent complications of endometriosis and adverse fertility outcomes.