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Pentalogy of Cantrell is a rare syndrome characterized by 5 distinctive anomalies: epigastric omphalocele, sternal cleft, diaphragmatic defect, diaphragmatic pericardial defect, and intracardiac defect. There are limited case reports documented since its first description in 1958 due to either underdocumentation or underrecognition. One possibility for underrecognition is the idea that there can be incomplete expressions of the syndrome, and these cases may be reported as separate diagnoses. This article explores the case of an infant with the prenatal diagnosis of giant omphalocele who was found to have a complete expression of the pentalogy. Infants with diagnoses such as giant omphalocele and diaphragmatic hernia require complex medical and surgical care. Nursing is in the perfect position to provide these infants with safe, quality, and interdisciplinary care and to provide vital, comprehensive teaching to their parents and families. The need for and value of comprehensive follow-up of these infants cannot be expressed enough.
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