We present a case of recurrent strokes in a patient with absent left internal carotid artery (ICA) and pulmonary arteriovenous malformation. Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between pulmonary artery and pulmonary vein, cause extracardiac right to left shunting of blood and are known to significantly increase the risk of stroke primarily due to paradoxical embolization. They are often hereditary and are commonly associated with hereditary hemorrhagic telangiectasias (HHT). Delayed bubbles seen in the left ventricle (after 3 cardiac cycles) on transthoracic echocardiogram with bubble study is often the first clue to the presence of PAVMs. CT scan of the chest can confirm the diagnosis. Percutaneous embolotherapy is the treatment of choice with reduction in stroke risk post embolization.