The origin of the right coronary artery from the left aortic sinus is an uncommon anomaly, which has been shown to be a cause of sudden cardiac death in young and adult patients, often in association with physical exertion. So far, to the best of our knowledge, only six cases of sudden death related to this anomaly have been reported in newborns and infants. We describe here a further case which reinforces the need of precise postmortem examination in these fatalities.