AbstractThe combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called Herlyn‐Werner‐Wunderlich 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 Ladd'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.