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Introduction:  Acinar cystic transformation (ACT) of the pancreas is characterized by multiple cysts lined by dual ductal and acinar-type of epithelium. ACT is typically a disease of adulthood and has not been described in a neonate. Case report: Autopsy of this term 3-day old male demonstrated cystic transformation of the entire pancreas measuring 42 mm in its largest dimension. The main pancreatic duct was patent. The numerous variable-sized cysts were lined by both ductal (CK7-positive) and acinar (trypsin-positive) epithelium. Congenital hemochromatosis of the liver, complete proximal jejunal atresia, gangrene of the post-atretic jejunum, and subglottic stenosis were associated features. Discussion/Conclusion: ACT may occur in the neonate in association with other abnormalities.