A persistent fifth aortic arch was recognized in a 4-year-old boy by echocardiography, magnetic resonance imaging and angiocardiography. The case was unique in at least two respects: right-sided aortic arch, and systemic-to-pulmonary connection without intracardiac lesion. This rare type of anomaly was correctly diagnosed and successfully repaired by patch closure of the arch. This is the first report of this combination of lesions in the Orient.