We report the clinical case of 23-year-old patient with liver cirrhosis of unknown genesis, significant resistant ascites, and 2 episodes of bleeding from esophageal varices. Evaluation did not find any cause of liver disease, and the patient was placed on the transplant wait list due to subcompensated liver function (Model for End-Stage Liver Disease score of 16, Child-Pugh class B) and poorly controlled severe portal hypertension. After treatment with diuretics, largevolume paracentesis, antibiotics, and vasoconstrictors, hepatorenal syndrome and spontaneous bacterial peritonitis resolved and liver function improved significantly. Because the patient showed consistently good liver function and resistant portal hypertension, liver transplant was delayed with decision to perform transjugular intrahepatic portosystemic shunting instead. During the attempt of shunting, occlusive thrombosis of the iliac veins, inferior vena cavae, and hepatic veins were diagnosed and the procedure was stopped. Therefore, considering preserved liver function and severe portal hypertension, diagnosis of Budd-Chiari syndrome with subsequent development of liver cirrhosis was made. The patient was recommended to undergo evaluation to exclude thrombophilia as a cause of thrombosis.