We present the case of a 26-day-old female in whom left aortic arch with right-sided descending aorta occurred. The previously healthy newborn baby was found dead on her bed. This anomaly is very uncommon and its clinical presentation as sudden death is also exceptional with only a few cases having been reported in the literature. The pathological findings and embryological origins of this malformation, so-called 'circumflex aorta' are discussed. The pertinent literature is reviewed.