A 54-year-old man with type C cirrhosis was admitted to another hospital complaining of hematemesis due to rupture of the esophageal varices and underwent hemostasis with endoscopic variceal ligation (EVL). Abdominal ultrasonography revealed ascites, and color Doppler ultrasonography showed IAPF between the branch of the left hepatic artery and umbilical part of the left branch of the portal vein. The right portal venous flow was hepatopetal, and the left portal venous flow was hepatofugal. Contrast-enhanced computed tomography (CT) demonstrated IAPF in the left lobe, and the umbilical part of the left branch of the portal vein was enhanced simultaneously in the arterial phase. Digital subtraction angiography (DSA) revealed diffuse IAPF and an early filling of the left branch of the portal vein. The cause of portal hypertension was IAPF supplied by A2, A3, and A4, and transcatheter arterial embolization (TAE) using microcoils was performed to close the fistula. A2, A3, and A4 were embolized; however, the fistula was not completely occluded. Thereafter, there were a total of four hematemeses due to esophageal variceal rupture, and a total of six EVLs were performed. The second TAE also failed to reach complete occlusion because of diffuse collateralization. As hematemesis was repeated after treatment, the patient was transferred to our hospital for further treatment. Laboratory results were as follows: white blood cell count of 4500/μL (normal, 4000–9000); red blood cell count of 328 × 104/μL (normal, 427–570 × 104/μL); serum hemoglobin concentration of 10.2 g/dL (normal, 14–18 g/dL); serum platelet count of 12.8 × 104/μL (normal, 15–35 × 104/μL); aspartate transaminase concentration of 69 IU/L (normal, 8–38 IU/L); alanine transaminase concentration of 45 IU/L (normal, 4–44 IU/L); serum albumin concentration of 4.1 g/dL (normal, 3.9–4.9 g/dL); total bilirubin concentration of 0.6 mg/dL (normal, 0.2–1.2 mg/dL); prothrombin time of 67.0%; and ICGR15 level of 12.4%. The clinical Child-Pugh classification status was B. As with previous hospital examinations, abdominal CT demonstrated ascites and remaining IAPF in the left lobe of the liver. Although left hepatectomy including IAPF was thought to be needed, we concluded that major hepatectomy at this point had a high risk because of poor general condition due to frequent hematemesis and deterioration of liver function. Although an apparent decline in liver function due to frequent massive bleeding was possible, the general condition was extremely poor and was not suitable for left hepatectomy. Therefore, we performed ligation of the draining left portal vein and dissection of the left gastric vein that supplied varicose veins. A catheter was inserted from the paraumbilical vein to measure the portal venous pressure. Portal venous pressure decreased from 330 to 210 mmH2O after ligation of the left portal vein. The operating time was 251 min, and the intraoperative bleeding was 340 mL. However, melena appeared on the 5th postoperative day, and the progression of anemia was observed. An emergency upper gastrointestinal endoscopy was performed on suspecting bleeding from the esophageal varices. Although there was no active bleeding, EVL was performed for the esophageal varices with red color signs. The laboratory results on the 14th postoperative day were as follows: aspartate transaminase concentration, 48 IU/L; alanine transaminase concentration, 34 IU/L; serum albumin concentration, 3.6 g/dL; total bilirubin concentration, 0.5 mg/dL; prothrombin time, 67.9%; and ICGR15 level, 13.8%. Ascites disappeared at the CT findings in the postoperative course, and the clinical Child-Pugh classification status improved from grade B to grade A. After the first surgery, the general condition and liver function were improved on the 14th postoperative day. Therefore, left hepatectomy was performed to remove the IAPF completely on the 21st postoperative day. Adhesion was observed around the hepatic hilum because of the first operation. Furthermore, the division of the hepatic hilum was hemorrhagic owing to portal hypertension. As the left portal vein was ligated at the time of the first operation, the demarcation line was found on the liver surface by dissection of the left hepatic artery. After mobilization of the left liver, parenchymal dissection was performed under intermittent inflow occlusion, that is, 15 min of occlusion followed by 5 min perfusion. The operating time was 318 min, and the intraoperative bleeding amount was 1800 mL. In the macroscopic findings of the resected specimen, arterioportal fistula could not be identified. In the microscopic findings, the background liver tissue showed the presence of many pseudo-nodules, indicating liver cirrhosis. Many dilatated vessels in Glisson’s sheath and arterioportal fistula were observed. Contrast-enhanced CT after left hepatectomy revealed that earlier enhancement of the branch of the portal vein disappeared in the hepatic arterial phase. Although anorexia and wound infection were noted, there were no other major complications, and he was discharged on the 32nd postoperative day. There was no recurrence of portal hypertension for 1 year and 3 months after hepatectomy.