This is a case report of an operation for the pulmonary arteriovenous fistula supplied from the systemic arteries instead of the pulmonary artery. The operation of systemic arteriovenous pulmonary fistula has formidable technical challenges due to its extensive collateral circulations. A 16 year-old female patient, diagnosed as systemic arteriovenous fistula with multiple tortuous feeding vessels and with hereditary hemorrhagic telangiectasia, was initially managed with arterial embolization before the operation. A 15${ imes}$8cm sized huge vascular malformation was removed by RML and RLL bilobectomy. During the operation, we encountered annoying massive bleeding and pulmonary congestion originated in its extensive collateral circulation. The patient was discharged after conservative management without specific problem on the 15th postoperative day. For the safe operation as well as good operative result, it seemed that meticulous ligation of the multiple collateral vessels should be performed prior to that of pulmonary veins.