In pulmonary arteriovenous fistula (AVF) there is direct communication between the pulmonary arteries and the pulmonary veins without intervening capillary system. The clinical pictures of this anomaly depends on the size and number of pulmonary artery fistula. Common complaints are easy fatigability, headache, epistaxis and hemoptysis due to associated vascular anomalies like hereditary hemorrhagic telangiectasia. Clinical examination shows cyanosis, clubbing, cutaneous telangiectasia, no cardiomegaly, no cardiac murmur but continuous murmur may be present over lower part of the chest. Chest X-ray shows multiple pulmonary opacities. Echocardiographic appearance of contrast in left side of heart, 3-4 cardiac cycles after venous injection gives the diagnosis. Pulmonary angiography (conventional / CT / MRI) confirms the site, number, size and the feeding artery. Nonsurgical (coil closure) or surgical measures are advised when fistula is large.