A 65-year-old italian patient developed complicated portal hypertension immediately after orthtopic liver transplantation (OLT) necessitating shunt creation. One to five weeks after OLT, massive ascitic fluid losses of up to 121/day developed. Vascular and major hepatic-parenchymal abnormalities were excluded by duplexsonography, angiography and initial histology, respectively. A peritoneovenous shunt (Denver-shunt) on day 31 after OLT reduced (by about 50%) but did not stop ascitic fluid losses. Furthermore, three variceal bleedings occurred after implantation of the Denver-shunt. Direct portography on day 45 after OLT revealed portal hypertension (pressure gradient of 26 mmHg) requiring the implantation of a transjugular intrahepatic portosystemic stent-shunt (TIPS) leading to a reduction of the pressure gradient to 13 mmHg. Subsequently, ascites resolved within ten days and esophageal varices improved. Liver function parameters normalized inspite of recurrence of HCV infection with detection of HCV RNA in serum already in the fifth week after OLT. During follow-up, histological findings deteriorated from mild changes to extended fibrosis at day 61 after OLT, which might have contributed to the maintenance of portal hypertension. The deterioration of liver histology was accompanied by an improvement/normalization of liver graft function. There was no evidence for additional viral liver infections, e.g. hepatitis B or cytomegalovirus infection. This case illustrates an etiologically unclear syndrome developing directly after OLT and reaffirms the effectiveness of TIPS in the treatment of complicated portal hypertension even after liver transplantation.