A large shunt between the inferior mesenteric vein (IMV) and the inferior vena cava (IVC) is a rare type of portosystemic shunt in patients with hepatic encephalopathy. We report a patient with hepatic encephalopathy due to a large IMV-IVC shunt who was successfully treated by balloon-occluded retrograde transvenous obliteration. The procedure involved a combination of 11 metallic coils and 5 ml of 5% ethanolamine oleate with iopamidol as the sclerosing agent. After complete obliteration of the shunt, his symptoms disappeared. At 2-years follow-up he was free of clinical symptoms, the size of his liver had slightly increased, and his liver function was preserved.