Severe hepatic veno-occlusive disease is still a potentially lethal complication after bone marrow transplantation. We here report the case of a patient who developed liver veno-occlusive disease with severe hemodynamic dysfunction and was successfully treated by means of a trans-jugular intrahepatic portosystemic shunt. After three years, he is still disease-free with a functioning shunt and a normal laboratory liver profile. A trans-jugular intrahepatic portosystemic shunt is a treatment option that has been used in very few patients affected by hepatic veno-occlusive disease; its indications and timing remain to be established.