Background : Pentalogy of Cantrell is a very rare congenital anomaly which is diagnosed with severe thoracoabdominal defect, omphalocele, lower sternal defect, anterior diaphragmatic defect anterior pericardial defect and ectopia cordis.Pathogenesis is unknown, mesodermal developmental defects in 14-18 days after conception maybe responsible . Case Report: The case is newborn with omphalocele and ectopia cordis. Conclusion: All newborns with omphalocele must be evaluated for Pentalogy of Cantrell and accompanying anomaly.

Keywords: Pentalogy of Cantrell, Omphalocele, Ectopia cordis, Khorramabad

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