Pancreatoblastomas are rare tumours, which usually occur in childhood. Here we describe a pancreatoblastoma in a 39-year-old woman. The tumour was located in the tail of the pancreas and consisted of cells forming well-differentiated acinar structures and scattered solid components ("squamoid corpuscles"). Immunocytochemically, the acinar components were positive for pancreatic enzymes and pancreatic stone protein, while the cells of the "squamoid corpuscles" lacked these markers. There was no p53 overexpression nor any mutation at codon 12 of the Ki-ras oncogene. The main differential diagnosis of this tumour was acinar cell carcinoma, because both tumours have a number of features in common (scattered solid components, positivity for pancreatic enzymes, lack of p53 overexpression and of Ki-ras mutation). Findings which distinguished the pancreatoblastoma and separated it from acinar cell carcinoma were the negativity of the solid components ("squamoid corpuscles") for neuroendocrine markers and their very weak keratin positivity. As the patient is alive and well 30 months after tumour resection, this pancreatoblastoma also differs in biology from the usual acinar cell carcinoma.