Primary epithelial tumors of the pancreas are extremely uncommon in children, and among these, acinar cell carcinoma (ACC) is the most rare. Here we describe our recent observations in the case of a 10-year-old boy with one of these exceptional examples. The histologic diagnosis of ACC was supported by both immunohistochemistry and electron microscopy. Despite its rarity, ACC should be kept in the differential diagnosis of pediatric pancreatic exocrine tumors. We also provide a comparison with an example of solid pseudopapillary tumor, another relatively infrequent epithelial tumor of the pancreas in the young. We review the relevant literature addressing the clinical and pathologic features of ACC and its distinction from other pancreatic neoplasms.