Persistent fifth aortic arch has been thought to be rare but often relevant to systemic circulation, however when it connects to the pulmonary artery (PA) in pulmonary atresia, it may be the sole arterial supply to the lung. This report describes a case of rare arch anomaly including right aortic arch (RAA), PA sling in conjunction with persistent left fifth aortic arch (PLFAA) and left subclavian artery arising from the left fourth arch. The tetralogy of Fallot, pulmonary atresia, and total anomalous of pulmonary venous connection (cardiac) were also diagnosed. A neonate was referred to our hospital for surgical treatment of cardiac and extracardiac anomalies. Persistent fifth aortic arch connecting with pulmonary artery was initially thought to be patent ductus arteriosus (PDA), so prostaglandin E 1 administration was commenced. He underwent emergency colostomy for anal atresia. Subepiglottic tracheal stenosis was diagnosed at initiation of anesthesia. At age 1-month-old, he required systemic to pulmonary shunt and reimplantation of left pulmonary artery through a median sternotomy using extracorporeal circulation. At the operation the PDA was divided and oversewn, and the wall structure was the same as that of a normal artery. The left pulmonary artery behind trachea was dissected and we then cut away and reimplanted to pulmonary trunk. Tracheostomy was performed at the age of two months. With the technical development of diagnostic imaging, the morphological features of arch anomaly were clearly demonstrated, but some understanding of embryological aspects are still required for diagnosis.