UNLABELLED: The relation of hemophilia A with thrombophilia V Leiden is extremely rare in the literature. Furthermore, hemophiliac patients have an increased risk of severe life-threatening hemorrhage, blood transfusions, and therefore hepatitis transmission, mainly hepatitis C (HCV).AIMS AND METHODS: We present a 54-year-old male with a 5-year history of decompensated liver cirrhosis on the grounds of HCV hepatitis, hemophilia A, and thrombophilia V Leiden. He was admitted to our department because of severe abdominal distension, resembling 'tense ascites' despite the use of diuretics. Clinical examination showed shifting dullness and a protuberant abdomen, while hematological and blood chemistry results revealed thrombopenia (platelets: 77000/mL) and hypoalbuminemia. Repeated abdominal paracentesis (under factor VIII administration) failed to remove ascitic fluid, while abdominal echosonography and computed tomography revealed severe edema of mesenterium and intraabdominal viscus and the absence of free ascitic fluid, atrophic cirrhotic liver, and splenomegaly. Moreover, abdominal doppler echosonography revealed signs of portal hypertension, previous portal vein thrombosis, and revascularization of the portal vein. Gastroscopy showed esophageal varices grade II, without signs of bleeding. A-FP and all other laboratory examinations were normal.RESULTS: Our patient was intravenously treated with albumine and diuretics (furosemide) with mild improvement of his abdominal distension. During his hospitalization he presented an episode of spontaneous bacterial peritonitis and hepatic encephalopathy, which were successfully treated with lactulose clysmas and ciprofloxacine. He was discharged in a good general condition.CONCLUSION: According to our case we consider the false clinical picture of 'tense ascites' of our patient as a rare clinical presentation of decompensated liver cirrhosis, with severe edema of mesenterium and viscus, on the grounds of preexisting portal vein thrombosis, in a patient with combined hemophilia A and thrombophilia V Leiden.