A case report of a neonate with pulmonary arteriovenous malformation is described. The anomaly was prenatally diagnosed, and family history was positive for Rendu-Osler-Weber syndrome. Because neurologic symptoms developed during the second week of life, surgical resection was deemed the best curative option to avoid further embolic events and reoccurrence of the lesion. Previous reports detailing coil embolization therapy for pulmonary arteriovenous malformation are discussed.