Obstructed hemivagina and ipsilateral renal agenesis with intestinal malrotation

The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called H erlyn‐ W erner‐ W underlich syndrome ( HWWS ) or obstructed hemivagina and ipsilateral renal anomaly ( OHVIRA ) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal non‐rotation, anomalies of the large vessels of the abdomen including duplication of the inferior vena cava and a high‐riding aortic bifurcation, and hypodontia. Hypodontia has never been reported in a patient with HWWS . The patient underwent a preventative L add's procedure and vaginal reconstruction. To prevent serious complications from concomitant anomalies such as intestinal malrotation, a patient with HWWS should be evaluated in detail for associated malformations.