Objective: To determine the clinical pathological features, pathological diagnosis, and differential diagnosis of the pancreatoblastoma (PBL) in infants. Methods: Pathological and immunohistochemical observations were conducted in 2 infants with PBL, and their clinical data and literature were analyzed and reviewed, respectively.Results: These 2 infants showed a feature of abdominal mass. Microscopic findings revealed that the tumors were composed of epithelial cells with amount of stroma. The epithelial cells were separated by fiber stromal tissues, forming nest or organoid patterns. The tumors exhibited acini, short tubules and solid islands. The tumor cells in the solid islands formed squamoid nests. Immunohistochemical results showed that the tumor cells were positive for cytokeratin (n=2), α-1-antichymotrypsin(AACT)(n=2), neuron specific endolase (n=1), chromogranin A (n=1), CD99 (n=1), and carcinoembryonic antigen (n=2), but negative for synaptophysin and alpha-fetoprotein. Conclusion: PBL is a rare lower grade pancreatic malignant tumor and often occurs in infants. The squamoid nest is the characteristic of PBL, which should be differentiated from the other pancreatic neoplasms and teratoma.