Pentalogy of Cantrell is an extremely rare birth defect. It associates 5 malformations including: a supraumbilical hernia of the abdominal wall, a defect of the anterior part of the diaphragm and the diaphragmatic pericardium, an anomaly of the lower part of the sternum and cardiac malformations.
We most often find a notion of consanguinity of the parents. The long-term prognosis for children with this anomaly depends mainly on the complexity of the associated heart defect.
We report a case of pentalogy of Cantrell in a 2-day-old male newborn with extra thoracic cardiac ectopia.