Objective: To present a case of müllerian defects associated with other anomalies, including ipsilateral renal agenesia, duplication of inferior vena cava, high-riding aortic bifurcation, and intestinal malrotation. Design: Case study. Setting: Academic medical center. Patient(s): A 16-year-old woman with müllerian defects associated with vascular and intestinal anomalies. Intervention(s): Excision of vaginal septum. Main Outcome Measure(s): Improvement of symptoms caused by obstructed müllerian defect. Result(s): After an incision on the vaginal septum, a large amount of chocolate-colored fluid was drained, and the dimensions of the right uterus were diminished. Then the vaginal septum was excised completely; we saw a second distinct cervical portion. Conclusion(s): Uterus didelphys with obstructed hemivagina is almost always associated with renal agenesis ipsilateral to the obstruction. Inferior vena cava duplication associated with müllerian anomalies is an extremely rare condition. In addition, the presented case also had a high-riding aortic bifurcation and intestinal malrotation. To the best of our knowledge, this is the first case to include all of these anomalies. © 2008 American Society for Reproductive Medicine.