• Zlatan Zvizdic, Irmina Sefic-Pasic, Amira Mesic, Sabina Terzic, and Semir Vranic.
• Clinic of Pediatric Surgery.
• Medicine (Baltimore). 2021 Apr 9; 100 (14): e25470e25470.

RationalePentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior diaphragm, diaphragmatic pericardium, and various intracardiac defects.Patient ConcernsWe report a case of complete POC affecting only the first-born of a set of premature dizygotic twins.DiagnosisA giant omphalocele with an eviscerated liver and bowel on prenatal, obstetric ultrasonography at 24 gestational weeks was observed. At birth, physical examination confirmed a massive (10 × 8 cm) epigastric omphalocele in which a significant part of the liver was seen. A postnatal echocardiogram revealed the presence of an ostium secundum atrial septal defect, perimembranous ventricular septal defect, and moderate pulmonary stenosis. X-ray showed an abnormal intrathoracic positioned stomach, which was confirmed with a plain x-ray of the upper intestinal tract with hydrosoluble contrast. Computed tomography (CT) scan revealed the sternum's absence and a close connection between the pericardial sac and the stomach wall.InterventionsThe patient underwent surgical intervention at 18 days of age.OutcomesDespite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery.LessonsPOC is a complex, high-mortality syndrome whose management requires a multidisciplinary approach and meticulous planning. Despite all efforts, POC carries a poor prognosis, particularly in patients affected by its complete form.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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