A 37 year old man known to have inferior vena cava agenesis, presented to our center with severe symptoms of pelvic venous congestion. Surgical approach was attempted by creating a bypass between the right external iliac vein and the portal vein using an autogenous venous bypass (superficial femoral vein). Over a three year follow up, the bypass remained patent with complete resolution of symptoms. The ilio-portal venous bypass suggests a surgical alternative that has not been previously described.
