A 22-year-old male was found to have Budd-Chiari syndrome (BCS) with complete occlusion of hepatic veins and hepatic inferior vena cava (IVC). The patient underwent a direct intrahepatic-portocaval shunt (DIPS). Visualization of the portal venous system was made in a trans-splenic approach. A shunt was then successfully created between the distal-most IVC and main portal vein. The patient was later diagnosed with Behçet`s disease (BD). BD accounts for less than 5% of BCS cases. Although rare, these patients suffer from high mortality and high percentage of IVC occlusion. Although technically challenging, DIPS might be a good option for these patients.
