Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare cardiovascular disease presented with an incidence of 1 per 300000 live births.

In this article, we present four cases of ALCAPA in infancy. Two infants presented with respiratory distress and two with a heart murmur. Their coronary arteries were derived from their pulmonary arteries. Two of them died, one after surgical procedure and one before surgical intervention. Of remained cases, one of them had a good surgical outcome, and another was missed in the follow-up.
A brief review was done on case reports of ALCAPA in children. Eighteen articles were found, including 201 pediatric cases.

The vast majority of affected children will die within the first year of life if ALCAPA is not diagnosed and treated appropriately.