A 20-year-old patient underwent liver transplantation (LT) because of chronic hepatitis-associated cirrhosis. After transplantation, the patient developed partial portal vein thrombosis without deterioration of liver function. However, in the long-term follow-up, the patient developed refractory portal hypertension with recurrent episodes of severe upper gastrointestinal bleeding. Extensive nonoperative treatment including multiple esophageal ligatures and sclerotherapy was not successful. Thus, the patient had to be treated surgically with a mesocaval H-shunt. Pathophysiology, therapy and prognosis of this rare complication after LT are discussed with regard to the literature.