Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele—A Case Report

Ectopia cordis (EC) is a rare malformation due to failure of maturation of the midline mesodermal components of the chest and abdomen. It can be defined as 0.1% of congenital heart diseases, and it could present isolated or could belong to the spectrum of the Pentalogy of Cantrell (PoC), which is a rare congenital disorder first described in 1958 by Cantrell.We are reporting a rare case of total ectopia cordis, associated to a major omphalocele, total agenesis of the sternum, anterior diaphragmatic deficiency, absence of pericardium, and persistence of the Ductus arteriosus, making therefore these features compatible with a full spectrum of the Pentalogy of Cantrell, encouraging us to report this case.