The patient was a 73-year-old male who was identified with an increase of serum PIVKA-II during a treatment for chronic hepatitis B. Hepatocellular carcinoma (HCC) of 60 mm in diameter with satellite nodules was diagnosed in segment 8 of the liver. In addition, portal vein tumor thrombosis (PVTT) of the right branch (Vp3) and metastases to bilateral lung and right adrenal gland were recognized. He received serial treatments with transcatheter arterial chemoembolization (TACE), radiation therapy and hepatic arterial chemotherapy with reservoir for primary liver tumor and PVTT. Soon after the treatments, PVTT was reduced in size and the serum level of PIVKA-II was decreased to 57 mAU/ml. After three months, the level of PIVKA-II had increased again and the size of the right adrenal metastasis grew to 50 mm in diameter. He received TACE to the right adrenal metastasis and percutaneous transhepatic portal chemoembolization to prevent further growth of PVTT. In spite of several treatments, the therapeutic effect was insufficient. Therefore, we performed right adrenalectomy and radio-frequency ablation of HCC in the liver S8. After the surgery, he received two times of TACE and the viable tumor had disappeared on CT and MRI. Prognosis of HCC with PVTT and distant metastasis is very poor. The two-year survival rate is less than 10%. However, it is possible to improve the prognosis of advanced HCC by multidisciplinary treatment with surgical intervention, local chemotherapy and radiation therapy.