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Gastroschisis complicated by intestinal atresia is a complex problem. Six cases are presented. A review of the literature and of our own cases shows a high mortality rate. Success or failure is related more to the pathology present than to any specific method of operative management. Resection and primary anastomosis is the favored method of treatment, as the intestine heals well in spite of its appearance. Primary closure of the abdominal wall musculature and skin is done whenever possible. A gastrostomy is used uniformly. Intravenous hyperalimentation is critical to survival of these babies and should be used early. The use of this therapeutic modality allows for the onset of gastrointestinal function spontaneously (often over prolonged periods of time) without nutritional deprivation. Intestinal atresias almost always are easy to identify in babies with gastroschisis. Extensive dissection and mobilization of this friable intestine is contraindicated in those babies in whom an atresia is not obvious but only suspected. In such cases the gastroschisis defect should be managed by whatever method is deemed appropriate and the baby observed while receiving intravenous nutritional support. If an atresia is present, it can be managed later in the baby's course by resection and primary anastomosis.