Portal hypertension often complicates extrahepatic biliary atresia and may result in life-threatening variceal bleeding. in more than 30% of cases, variceal bleeding cannot be controlled sufficiently by medicinal treatment and sclerotherapy. In these cases a portosystemic shunt can reduce portal hypertension. The transjugular intrahepatic portosystemic shunt (TIPS) is implanted using interventional radiological techniques. In comparison to a surgical portosystemic shunt, this method has a reduced mortality, and liver transplantation can be performed in patients with TIPS without impairment of transplant function and overall prognosis. The method is well established in adult patients but there is only little experience concerning TIPS implantation in children. We present a girl with extrahepatic biliary atresia. Ar the age of 8(6/12) years the patient presented with variceal bleeding refractory to sclerotherapy. Since no donor organ was available at that time TIPS was implanted. The procedure was performed successfully at the second attempt and portal hypertension significantly reduced. Liver transplantation was performed without complications three months later. Discussion: We suggest that TIPS should be the method of choice in the treatment of variceal bleeding refractory to sclerotherapy in children waiting for liver transplantation.