Acinar cell carcinomas and pancreatoblastomas are malignant tumors of the pancreas, showing predominantly acinar differentiation characterized by the immunohistochemical expression of pancreatic enzymes. Histologically, they usually display acinar and/or solid patterns, but may occasionally also exhibit cystic structures. The key feature of pancreatoblastomas is the presence of squamoid corpuscles. Acinar cell carcinomas predominantly occur in adults, pancreatoblastomas in children. Both tumor types commonly show allelic losses on chromosome 11p and mutations in the APC/beta-catenin signaling pathway. Pancreatoblastomas, in contrast to acinar cell carcinomas, are potentially curable.