Pulmonary arteriovenous fistula is relatively rare disease, and the most common types are congenital in origin. The clinical presentation of pulmonary arteriovenous fistula ranges from incidental finding on a chest roentgenogram to polycythemia, cyanosis, congestive heart failure, and major neurologic deficits caused by paradoxical embolism. Recently we have experienced a case of asymptomatic, well-localized pulmonary arteriovenous fistula In a 13 years-old female patient, which was successfully treated by thoracoscopic lobectomy.