Exocrine pancreatic insufficiency has been described in 2 previous cases7,9,26; however, the diarrhea did not abate with either pancreatic enzyme replacement or total parenteral nutrition with bowel rest.7 Similar findings have been observed in a 2-year-old with X-linked lissencephaly with abnormal genitalia.10 In this case, vasoactive intestinal peptide was normal, and the administration of octreotide was effective in relieving the diarrhea. Here, VIP is linked to exocrine pancreatic insufficiency.