We report the case of a 4-year-old boy with coarctation of the aorta resulting from persistent fifth aortic arch, a rare abnormality, along with an interrupted fourth aortic arch. When he visited a general practitioner with an upper respiratory infection, a heart murmur was noted. Computed tomography findings led to the diagnosis of persistent fifth aortic arch with an interrupted fourth aortic arch. He underwent aortic arch repair, excision of the fifth aortic arch, and anastomosis of the original arch with the descending aorta. Pathologically, the tissue of the fifth aortic arch was different from that of the normal aortic arch.
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