Background: Pulmonary arteriovenous malformation (PAVM) is a rare structurally abnormal communication between the pulmonary arterial and pulmonary venous network, creating a pathologic intrapulmonary right-to-left shunt.

Methods: A four-year-old boy presented with the history of chest pain, cyanosis, respiratory distress, cough and hemoptysis for two years. He was diagnosed to have a congenital pulmonary arterio-venous malformation occupying the posterior segment of the right upper lobe. The data and photos were collected from hospital records with the consent from the parents of the patient. Ethical aspects were discussed by our departmental review board.

Results: CT Angiogram revealed that a large vascular mass in the posterior segment of right upper lobe. The lesion was supplied by branches from pulmonary arterial system as well as descending thoracic aorta. A joint team of cardiac and thoracic surgeons opened the chest by a posterolateral incision along the right 4^th intercostal space. A large AVM mass, measuring approximately 10 cm x 6 cm x 5 cm, was seen occupying the lower part of the upper lobe of right lung. Following ligation of the feeding vessels, the AVM had shrunk and the affected part of the upper right lobe could be removed with the help of a stapler gun. Immediately after resection, the saturation went up to 100%. He made an uneventful recovery and was discharged from the hospital on the 8^th postoperative day. Histopathologic examination confirmed diagnosis of AVM.