Background. Budd-Chiari syndrome (BCS) represents partial or total occlusion of the hepatic veins with or without simultaneous obstruction of vena cava inferior (VCI). The symptoms of BCS are abdominal pain, hepatomegaly, ascites, varices of the abdominal wall, sometimes bleeding from the upper part of gastointestinal tract (GIT), lower limbs swelling and jaundice. Primary BSC is a relatively rare condition occurring in one per 100 000 of the population worldwide. Case report. A male patient, 25-year-old, facing tooth postextraction complications, was presented with acute BCS. On admission, physical examination revealed pale-grayish complexion, more pronounced veins over the thorax and abdomen, ascites, enlarged liver rising 8 cm below the right costal arch and having a minor pleural effusion by the right side. The patient was submitted to Doppler sonography and computed tomography (CT) that verified the right leg deep veins thrombosis, as well as the presence of a thrombus in the intrahepatic portion of the VCI. Multislice computed tomography (MSCT) showed occlusion of hepatic veins (Budd-Chiari syndrome) and thrombosis of the VCI in the retrohepatic part 6 cm long. Also, increased values of transaminases and gamma GT and reduced values of albumines and serum ferrum were registered. Molecular examination revealed Factor V Leiden mutation - heterozygote. After preoperative preparations a mesocaval shunt was made using Gore- Tex ring graft of 12 mm. Intraoperatively, the blue enlarged liver was found with almost black zones of tense capsule. After a graft making, liver congestion decreased followed by the change of colour and volume. Within postoperative course metabolic and synthetic liver functions were obvious. Conclusion. In patients with BCS medicamentous treatment does not yield adequate results, but even causes worsening of general condition. Surgical therapy in the presented patient was performed timely regarding the stage of the disease due to which irreversible liver changes were prevented while decompression of the portal system provided time overbridging up to liver transplantation.