Diagnostic Challenges of OHVIRA Syndrome—A Case Report

<b>Background and Clinical Significance</b>: Herlyn-Werner-Wunderlich (HWW) syndrome, also known as OHVIRA syndrome (Obstructed HemiVagina and Ipsilateral Renal Anomaly), is a rare congenital anomaly of the female urogenital system characterized by uterine duplication, unilateral vaginal obstruction, and renal agenesis on the same side. The condition often remains undiagnosed until adolescence, when it presents with palpable pelvic mass, dysmenorrhea, and chronic pelvic pain. <b>Case report</b>: We present the case of a 13-year-old female patient diagnosed with OHVIRA syndrome following imaging studies. Surgical treatment involved incision of the vaginal septum and evacuation of accumulated blood, leading to symptom resolution and restoration of reproductive tract patency. <b>Conclusions</b>: This article discusses the clinical characteristics, diagnostic challenges, and the importance of early surgical intervention, emphasizing the necessity of considering this syndrome in the differential diagnosis of adolescent females with cyclic abdominal pain and renal anomalies. Early diagnosis and treatment can prevent severe health complications and improve both patients' quality of life and fertility.