A case of pancreatic polypeptide cell hyperplasia in a 76-year-old man who presented with subacute bowel pseudo-obstruction is reported. A computed tomography scan incidentally showed a pancreatic head lesion that was resected by pancreaticoduodenectomy. Histological examination showed expansion of the endocrine pancreas with increased numbers of pancreatic polypeptide cells in irregularly enlarged islets, ragged endocrine cell clusters, ductulo-insular complexes and microadenomas. The clinicopathological features of this rare and poorly understood condition are discussed.