Cantrell syndrome is a rare syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and the heart. The spectrum of anomalies varies widely. Less than 160 cases have been described in the world literature. We reported a premature infant, with the syndrome. The case with a rare congenital malformation consisting of a pentad of findings: ectopia cordis and absent pericardium and a midline supraumbilical wall defect, evisceration of the intestines and liver, and short sternum. We presented this case because of its rarity and discuss the pathologic findings.