Congenital arteriovenous malformations are rare vascular anomalies in infancy and childhood. In most cases, these lesions consist of dilated vessels that appear as a large plexiform mass of vascular channels with direct communication between the pulmonary arteries and the pulmonary veins. Authors of the article present a case report of a newborn with a congenital arteriovenous fistula in the lower lobe of the right lung. They demonstrate various types, etiology, macroscopic and microscopic findings in this anomaly.