An isolated carotid artery is a rare aortic arch anomaly. Instead of connecting to the aorta, the isolated carotid artery connects anomalously to the pulmonary artery. Chronically altered cerebral circulation poses the risk of cerebral hyperaemia following surgical reimplantation. We describe successful reimplantation of the isolated left common carotid artery in a child, highlighting the importance of a multidisciplinary approach for good clinical and neurological outcomes. We also briefly discuss the embryologic basis of this rare arch anomaly.