To evaluate the utility of echocardiography (echo) in the diagnosis of persistent fifth aortic arch (PFAA), a very rare congenital aortic arch anomaly, and to compare echo and computed tomography angiography (CTA) imaging findings to improve our understanding of this anomaly.Data on the clinical diagnosis, imaging findings, and clinical management of PFAA were retrospectively analyzed in 10 suspected cases of PFAA admitted to our hospital between January 2012 and February 2017. We compared echo as a first line examination modality, and CTA and surgery results as the gold standard. Weinberg's classification was used to classify the type of PFAA.All patients (100%) received echo examination, eight patients (80%) received CTA examination, and four patients (40%) received sternotomy surgery; all recovered well after surgery. According to Weinberg's classification, 2, 6, and 2 cases (20%, 60%, and 20%) were classified as Type A, B, and C, respectively. Echo was able to diagnose 5 cases of PFAA (1 Type A case and 4 Type B cases) in the first instance. The diagnostic conformance rate of echo was 62.5% after comparisons with CTA and surgery results.The clinical manifestation of PFAA was atypical, and its diagnosis depended primarily on medical imaging. Echo has a relatively high diagnostic accuracy for PFAA, which is very valuable for its early detection.