Ventral wall defects are extremely rare anomalies that are likely caused by the failure of the ventral wall to close during week 4 of development. We report a case of severe thoracoabdominal wall defect including complete thoracic ectopia cordis and gastroschisis. This combination represents a novel constellation of findings in a single patient. This unique case further demonstrates an anatomically normal heart with age-appropriate development and an intact diaphragm. We review the literature of other reports and discussions of entities that share overlapping features with this case.
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