Abstract. We describe a newborn infant with a variant of Pentalogy of Cantrell and herniation of the liver through an anterior diaphragmatic defect. Pentalogy of Cantrell, also known as thoracoabdominal syndrome or Pentalogy syndrome, is a rare disorder consisting of defects of the ventral abdominal wall, anterior diaphragm, sternum and heart. Crescentic midline anterior diaphragmatic defects have been described in Pentalogy of Cantrell. Occasionally bowel gains entry through the defect and can lead to cardiorespiratory compromise. Herniation of liver into the thorax through a diaphragmatic defect leading to significant respiratory compromise has not been reported to date. We describe an interesting and challenging clinical scenario which required a late surgical repair of the diaphragmatic defect.