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Three types of persistent fifth aortic arch (PFAA) have been described; one of these (type 2) is characterised by atresia or interruption of the superior, embryonic fourth aortic arch and a patent inferior fifth arch. All brachiocephalic arteries arise from a common aortic trunk. We present a case of a female neonate with increasing tachypnoea and acidosis on the fourth day of life. The femoral arteries were not palpable. On auscultation there was a 4/6 harsh systolic murmur on the left thoracic side radiating to the back. The child was intubated and prostaglandin E1 (PGE1) administered. Angiography showed all brachiocephalic vessels originated from a single trunk (left). At the distal part of the PFAA a mild narrowing was observed. Close to the PFAA a tiny ductus arteriosus with left to right shunt was detected. When the PGE1 was stopped during cardiac catheterisation the pressure in the aorta descendens decreased from 83/51 (64) mm Hg to 49/42 (45) mm Hg and angiography (right) showed a moderate stenosis. PGE1 infusion was restarted. On the next day the patient underwent elective surgery. To our knowledge this is the first case describing PGE1 sensitive systemic circulation in a newborn with PFAA type 2.