Gastrointestinal stromal tumors (GISTs) are the most frequently encountered mesenchymal tumors that develop anywhere along the gastrointestinal (GI) tract, but they account for less than 1% of all GI tumors. However, GISTs can also develop outside the GI tract. They are referred to as extragastrointestinal stromal tumors (EGISTs) and represent around 5-10% of all GISTs. In this latter case, the tumor itself is not connected to the GI tract and can affect the omentum, mesentery, and retroperitoneum. EGISTs can also originate from the pancreas, but they are extremely uncommon. It is estimated that pancreatic EGISTs account for about 5% of all EGISTs, with around 30 cases being reported in the literature so far. The current report presents an extremely rare case of pancreatic EGIST encountered in a 53-year-old woman with no remarkable personal or family medical history. Surgical resection of the pancreatic head tumor through pancreaticoduodenectomy using Whipple procedure combined with negative resection margins and adjuvant Imatinib mesylate treatment led to a favorable postoperative evolution. The follow-up at six months after surgery did not reveal any malignant changes.