Pelvic pain and a missing kidney: Unveiling OHVIRA syndrome with a tubo-ovarian abscess

Herlyn-Werner-Wunderlich syndrome (OHVIRA syndrome) is a rare congenital anomaly involving the Müllerian and renal systems, classically presenting as uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Due to its rarity and variable presentation, the diagnosis is frequently delayed or missed, leading to complications such as hematocolpos, pelvic pain, and endometriosis. This case highlights a diagnostically challenging presentation of OHVIRA syndrome in a 46-year-old female, who was initially admitted for suspected renal obstruction but was later found to have renal agenesis and a tubo-ovarian abscess associated with OHVIRA syndrome. Ultrasound and CT revealed left renal agenesis and a complex pelvic mass, while MRI confirmed OHVIRA syndrome with uterus didelphys, an obstructed hemivagina, and an ipsilateral tubo-ovarian abscess. Imaging modalities, including ultrasound, CT, and MRI, played a pivotal role in recognizing the underlying congenital anomaly. This case underscores the importance of maintaining a high index of suspicion in cases of chronic and acute pelvic pain with renal anomalies and the indispensable role of radiology in the timely recognition of complex congenital disorders. Furthermore, it emphasizes the need for clinicians to consider congenital urogenital anomalies even in adult women, as delayed diagnosis can result in significant gynecological and urological complications.