A 63-year-old man was referred to our hospital for evaluation and treatment of severe dyspnea on exertion which had persisted for a few years. He presented with cyanosis and markedly clubbed fingers, and laboratory data disclosed hypoxemia, polycythemia, and liver dysfunction. A chest X-ray film showed increased vascular markings in both lower lung fields. Arterial blood gas analysis showed severe hypoxemia, with a PaO2 of 46 Torr and a PaCO2 of 31 Torr while the patient was breathing room air. The PaO2 increased only slightly with inhalation of 100% oxygen, which suggested the presence of a large R-L shunt. The hepatopulmonary syndrome was diagnosed. Angiography of the pulmonary artery revealed a large pulmonary arterio-venous fistula with markedly dilated arteries in both lower lobes. Transarterial embolization was done three times with a total of 62 metal coils. There were no complications. Embolization reduced the shunt from 56% to 31%, increased the PaO2, and relieved the dyspnea. Pulmonary artery embolization can be useful in treating pulmonary arterio-venous fistulas associated with the hepatopulmonary syndrome.