Arteriovenous malformation (AVM) of the pancreas is a rare condition that may cause severe gastrointestinal bleeding. We describe a 54-year-old man with a 7-year history of recurrent duodenal ulcer due to AVM in the pancreatic head. We recommended pancreatoduodenectomy because of recurrent haemorrhage from the duodenal ulcer, but the patient refused surgery on several occasions. He was admitted to our hospital complaining of severe upper abdominal pain radiating to the back and was diagnosed with acute pancreatitis. He agreed at that stage to the surgical treatment. The resected specimen contained a highly vascular malformation in the pancreatic head and ulceration in the adjacent descending duodenum. Histopathological examination revealed numerous vascular structures with dilated and tortuous vessels in the pancreatic head, confirming the presence of AVM. Moreover, oedema, inflammatory cell infiltration, haemorrhage and necrosis were evident, confirming the presence of acute pancreatitis.