The case of a 28-year-old man with acute Budd-Chiari syndrome due to veno-occlusive disease is reported. Transjugular intrahepatic portosystemic shunt (TIPS) was performed after upper gastrointestinal endoscopy, duplex sonographic and abdominal computed tomographic examination, inferior cavogram with hepatic venous catheterization, and transvenous biopsy. A 10-mm parenchymal tract was created. The patient did well after the procedure; ascites resolved and liver function improved markedly. The shunt has remained patent up to now for 6 months.