Although NSLCS has been considered to have a benign course, we have described an infant who had intrauterine intestinal perforation and meconium peritonitis associated with the NSLCS. Infants of diabetic mothers who are stillborn or who develop abdominal distention shortly after delivery should be suspected of having this complication of NSLCS. This disorder must also be considered in the differential diagnosis of infants who present with meconium peritonitis.