Liver herniation with gastroschisis is an uncommon occurrence that is associated with a poor prognosis. This report presents a single case of complex gastroschisis complicated by herniation of the left hepatic lobe. In the subject case, the abdominal wall defect was successfully closed by sequential closure with negative pressure wound therapy after the initial application of a preformed silo. As there are no established standards for the management of gastroschisis with liver herniation, there exists an opportunity for multicenter review to define approaches to optimize clinical outcomes with this complex congenital issue. As a result of the complexity and rarity of this congenital abnormality, reports with a positive prognosis carry clinical relevance.

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