Persistent primitive hypoglossal artery complicated by atrial septal defect and congenital intrahepatic shunts.

During early embryogenesis, anastomoses are formed between the carotid artery and the basilar or the vertebral artery, and subsequently, these anastomoses regress. In some cases, these anastomoses remain as persistent carotid-basilar or carotid-vertebral anastomoses. Atrial septal defect (ASD), a communication between the atria at the septal level, is a congenital heart anomaly. Intrahepatic venous shunts between the portal and hepatic veins are very rare and only some are considered congenital. We present the first case report of a patient with an ASD, a persistent primitive hypoglossal artery, and congenital portahepatic shunts.