A 15-month-old girl, who presented with biliary cirrhosis secondary to cystic fibrosis with refractory ascites and recurrent intestinal bleeding, underwent percutaneous transjugular intrahepatic portosystemic shunting. Immediately following the procedure the ascites disappeared and no further bleeding occurred. The stent shunt was patent on Doppler ultrasound until the 22nd day. The patient died on day 22 because of liver failure due to a low-flow syndrome with severe hepatic ischaemia, but with no recurrence of bleeding or ascites.