Primary ampullary neoplasms have origin in the ampulla of Vater, an anatomical structure where the common bile duct and the pancreatic duct join together as a common channel. It represents <0.5% of all gastrointestinal cancers and approximately 7% of all periampullary cancers. The adenocarcinomas arising in this region originate from different epithelial cellular constituents present at the site, the histopathological classification encompass: intestinal type, pancreaticobiliary type, and mixed type. Pancreaticoduodenectomy is the treatment of choice when there is an overt or highly suspicious malignant behaviour. We present here the case of a 74-year-old male patient who presented to our department for further investigation of obstructive jaundice and a pancreatic mass associated with a six-month history of significant weight loss and mild epigastric pain. Eventually, a total splenopancreatectomy was performed given the extension of structural anomalies of the organ secondary to an ampullary adenocarcinoma.