| Summary: | We report a case of a 32 week gestational age preterm monozygotic twin neonate with a rare variant of Pentalogy of Cantrell who presented with severe respiratory distress syndrome, possible pulmonary hypoplasia, large congenital diaphragmatic hernia, large omphalocele, and atrial septal defect. Pentalogy of Cantrell poses a unique challenge for both neonatologists and surgeons, as it has a high mortality and morbidity rate. Prognosis of newborns with Pentalogy of Cantrell depends upon severity of both cardiac and extracardiac defects, and other associated anomalies. This report describes an extremely rare variant of the Pentalogy of Cantrell spectrum in a patient with a co-existent twin – twin transfusion syndrome and transient hydrops fetalis in utero, who survived with favorable outcome. Keywords: Pentalogy of cantrell, Prematurity, Monochornionic twin, Giant omphalocele
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