Abstract. Inflammatory myofibroblastic tumor (IMT) is a rare condition of unclear etiology that is commonly observed in the lung but rarely in the pancreas. WHO classified IMT as a potentially malignant or aggressive tumor. In the present report, the case of a 69-year-old male patient with an IMT in the head of the pancreas, who experienced anorexia, nausea and vomiting, is presented. The patient's clinical symptoms were nonspecific, and the imaging findings revealed a hypovascularized pancreatic mass with stenosis of the descending duodenum. The electronic endoscopy findings revealed protruding lesions in the duodenal bulb and the descending duodenum. Biopsies of the mass were conducted with an electronic endoscope, but were not diagnostic. Subsequent duodenopancreatectomy aided in determining a pathological diagnosis of IMT, based on the histology and immunohistochemistry results. The patient experienced a recovery without further incident, as observed during a regular follow-up 3 years later. IMT in the head of the pancreas is rare, particularly in adults. In the present study, an extremely rare case of IMT involving the head of the pancreas in an adult patient is presented, and the therapeutic options for this condition are discussed.