Pulmonary arteriovenous malformations (PAVMs) are congenital defects in the form of an anomalous bridging between a pulmonary arterial and venous system that sidesteps the normal pulmonary capillary. This anomaly is usually associated with hereditary hemorrhagic telangiectasia, leftover small group are sporadic cases but may occur as an isolated anomaly or as multiple lesions. Rarely, such abnormalities can be acquired. Few cases are diagnosed in the neonatal period, as it will pass silent without symptoms. Clinical manifestations occur soon after birth as cyanosis, tachypnea, and an abnormal sound as bruit over the AVM; and the most important point is the clinical suspicion of diagnosis in absence of pulmonary parenchymal and cardiac disease that can explain the persistence of cyanosis. We report a successful Amplatzer vascular plug occlusion of isolated multiple left side PAVM in a neonate, which was done on two stages with a 6 months interval, with no complications. Over 2-year follow-up, the patient remained well with no symptoms.