A 54-year-old man was admitted to our hospital with the symptoms of palpitation, dyspnea, and tarry stool. Upper gastroduodenal endoscopy revealed submucosal lesions with vascular ectasia in the second part of the duodenum. Dynamic computed tomography (CT) detected a hypervascular lesion in the pancreatic head and the duodenum. Selective angiography showed proliferation of a vascular network and early filling of the portal vein at the early arterial phase. With a diagnosis of pancreatic arteriovenous malformation (AVM), we performed pylorus-preserving pancreaticoduodenectomy. At laparotomy, localized and meandering vessels were seen on the surface of the head of the pancreas. Histological examination showed dilated tortuous vessels accompanied by severed elastic fibers in the vessel media and blood clot formation. The incidence of pancreatic AVM remains extremely low, and recurrent gastrointestinal bleeding is a frequent complication. To prevent recurrent bleeding and progressive portal hypertension, surgery may be the definitive management of symptomatic AVM.