BACKGROUND Arteriovenous malformation is an unusual cause of gastrointestinal bleeding, particularly in the pancreas. A definitive treatment strategy is not yet established. CASE REPORT We present the case of a 37-year-old man with underlying hypertension and no significant family history who presented with a 3-month history of intermittent epigastric pains and unintentional weight loss of 5 kg in 2 months. The upper endoscopy showed a large duodenal ulcer, which was uncontrolled with a standard dose of proton pump inhibitors. An abdominal computed tomography scan with contrast was indicated and revealed an enhanced mass of 2.5×3.5×4 cm in size, located on the second and third parts of the duodenum and head of the pancreas, indicating an arteriovenous malformation. On day 10 of hospitalization, the patient suddenly had melena and a drop of hemoglobin level to 5.6 g/dL; angiography intervention was successful to control the bleeding. However, gastrointestinal bleeding recurred after 2 weeks, and the patient successfully underwent a Whipple procedure. CONCLUSIONS The diagnosis and therapeutic management of arteriovenous malformations are uniquely challenging; therefore, pancreatic arteriovenous malformations should be listed on the differential diagnosis, particularly in those cases with non-healing and large duodenal ulcers. Otherwise, early imaging modalities should be performed to confirm the diagnosis. In particular, angiography can temporarily control bleeding before proceeding with more definitive therapy.