We report a case of pulmonary arteriovenous fistula with multiple hepatic arterioportal shunts. A 23-year-old man was admitted for evaluation of a nodular shadow in the right lower lung field. Chest CT showed a homogeneous and well-defined nodular shadow in the right S7. 3-dimensional CT revealed an arteriovenous fistula composed of a feeding artery and a draining vein in the right S7. Abdominal enhanced CT revealed a diffusely heterogeneous pattern in the liver parenchyma and the portal vein staining diffusely at the late arterial phase. We diagnosed pulmonary arteriovenous fistula associated with multiple hepatic arterioportal shunts. Pulmonary arteriovenous fistula is often associated with hereditary hemorrhagic telangiectasia (HHT). This case did not meet the criteria for HHT at this time. However, we could not exclude the possibility of HHT because clinical manifestations of HHT become apparent after 40 years old among 10% of patients. The natural history and treatment of HHT are not clear. We concluded that we should be careful to detect possible manifestations as HHT when we diagnose and follow up patients with pulmonary arteriovenous fistula.