Pulmonary arteriovenous fistula is a rare congenital vascular malformation resulting from abnormal capillary development with incomplete formation of vascular septum normally dividing the primitive connections between the venous and arterial plexuses. Recently we have experienced a case of the bilateral pulmonary arteriovenous fistula in 7 years-old female patient. On admission, clinical manifestations were cyanosis of lips, clubbing and cyanosis of digits, and exertional dyspnea. The PO2 in arterial blood gas analysis was 43.3mmHg. In left upper and right lower lobe pulmonary arteriovenous fistulas were confirmed by bilateral pulmonary angiography. Left upper lobectomy and wedge resection of right lower lobe were performed respectively. Postoperative results were good.