Arteriovenous malformation (AVM) of the pancreas is an extremely rare disease. It may be asymptomatic, but more than half of the patients present with gastrointestinal bleeding. The most common cause of the gastrointestinal bleeding is variceal bleeding due to the portal hypertension resulting from AVM. Bleeding from a duodenal ulcer and AVM to the pancreatic duct are rare findings. Surgical excision is the treatment of choice, but when portal hypertension has developed, this cannot be corrected even after surgical resection. We experienced a case of recurrent duodenal ulcer bleeding that was due to arteriovenous malformation in the head of the pancreas in a 45 year old man. He was successfully treated with pylorus preserving pancreaticoduodenectomy. (Korean J Gastrointest Endosc 2005;31:353357)