This paper presents a very rarely encountered case of a 45-year-old female, admitted in our Surgical Clinic for upper digestive bleeding (repeated hematochezia). The upper endoscopy was negative, but the barium meal discovered an apparently extrinsic duodenal (D3) stenosis; abdominal ultrasound diagnosed a left liver mass suggesting a metastatic tumor. The hematochezia relapse, with hemodynamic instability imposed emergency surgery; on laparotomy, a bleeding tumor located on the duodenopancreatic region was discovered, and a pylorus-preserving pancreaticoduodenectomy (Traverso-Longmire) was performed. The histology and immunohistochemistry established the diagnosis of duodenal stromal tumor, CD34 and CD117 positive, with an estimated progression risk of 34%. The postoperative evolution was favorable, the patient being alive, four years after the surgery.