An alpha fetoprotein (AFP)-producing tumour occurring in the head of the pancreas of a 30-year-old woman is reported. Histological examination revealed a markedly solid proliferation of tumour cells with prominent nucleoli and occasional luminal structures, some of which contained mucinous material stained with mucicarmine and alcian blue. No squamoid corpuscles were recognized. Immunohistochemistry showed intense positivity for lipase trypsin, and AFP basically, and single cells were also positive for carcino-embryonic antigen, CA19-9, synaptophysin and neuron-specific enolase. Pancreatic hormone-positive cells were absent. Electron microscopical examination revealed numerous granules of variable sizes in the tumour cells, which were considered to be zymogen. The tumour is an acinar cell carcinoma with multi-directional differentiation including the ability to produce AFP. Among AFP-positive pancreatic tumours, acinar cell carcinoma and pancreatoblastoma seem to be the most frequent.