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A case of double aortic arch with an atretic left arch distal to the origin of the left subclavian artery was suspected by echocardiography (ECHO) in an asymptomatic 9-year-old girl and confirmed by magnetic resonance imaging (MRI). The authors report their ECHO findings, which could be an important diagnostic tool for symptomatic patients because confusion exists in the literature regarding the differentiation of the aforementioned rare malformation from a right arch with mirror image branching. The authors support their ECHO proposal with an explanation based on the hypothetical double aortic arch plan set by Edwards.