Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex Müllerian and Wolffian duct anomaly. It is also known as Herlyn–Werner–Wunderlich syndrome (HWWS). It includes unilateral renal anomalies and uterine didelphys. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. We report here a 16-year-old female presenting with progressive cyclical pelvic pain, where magnetic resonance imaging (MRI) suggested the diagnosis of the OHVIRA syndrome. She was managed by surgical resection of the septum and draining of the obstructed vagina.