Congenital localized absence of the skin (CLAS) has been observed in various subsets of inherited epidermolysis bullosa (EB). Pyloric atresia (PA) is a rare disorder that is associated with EB. Here, we describe three preterm male infants with PA, CLAS, and EB at birth, one of whom had multiple bullae and erosions on the stomach and pericardium. To the best of our knowledge, this is the first report of findings regarding a PA-EB-CLAS association.