Mucinous papillary tumors represent a clinicopathologic entity recognized with increasing frequency. We observed a 59 year old man affected by chronic pancreatitis, jaundice, and 10-fold increase of CA 19-9 with a large pancreatic mass. Partial gastrectomy, cholecystectomy + transduodenal papillostomy and pancreatic-jejuneal anastomosis were previously performed. The patient underwent total pancreatectomy. Perioperative morbidity was absent. Histology showed a diffuse non-invasive papillary mucinous tumor. No adjuvant treatment was required and five months after surgery the patient is alive and free of disease.