Cantrell's pentalogy is a congenital defect, described more than 50 years ago by Cantrell, Haller and Ravitch. Long term survival of patients with Cantrell's pentalogy is extremely rare. We report a case of pentalogy of Cantrell class 1, that showed sternal cleft, left anterolateral diaphragmatic hernia, thoraco-abdominal ectopia cordis, diaphragmatic pericardial defect, patent ductus arteriosus and a large omphalocele. Initial surgery was done in his first day of life, obtaining partial correction of the defect and allowing survival with good quality of life. At the age of 18 years, he showed an abdominal wall defect measuring 22x24 cm, with near-total eventration of the liver and small bowel. Surgical correction consisted on visceral partial reduction and abdominal wall closing with a microporous polytetrafluoroethylene mesh and dermal flaps. Judicious combination of reconstructive techniques obtained long term survival free of symptoms and excellent aesthetic results.
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