A Complex Case Report of OHVIRA syndrome: Uterine didelphys, obstructed hemivagina, and renal agenesis.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital anomaly resulting from abnormal development of the Müllerian and Wolffian ducts, characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We report the case of a 26-year-old woman who presented with chronic pelvic pain and leukorrhea; clinical examination revealed a palpable pelvic mass, and imaging confirmed the diagnosis of OHVIRA syndrome. MRI demonstrated a didelphic uterus, an obstructed hemivagina, and absent ipsilateral kidney. Although commonly diagnosed in adolescence after menarche, this case highlights a delayed presentation. Prompt diagnosis is crucial to avoid complications such as endometriosis or infection. MRI plays a key role in the diagnostic process and surgical planning. Treatment is based on relieving the obstruction and preserving fertility, ranging from septum resection in simple cases to hemi hysterectomy in complex anomalies. This case underscores the importance of early recognition of OHVIRA syndrome to prevent long-term morbidity and optimize reproductive outcomes.