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.Key words: Ectopia cordis, major omphalocele, sternum agenesis, anterior diaphragmatic deficiency, absence of pericardium, persistence of the Ductus arteriosus.
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