A man in his 60s with epigastric pain was diagnosed with acute pancreatitis and subsequently recovered following conservative treatment. However, because of repeated upper abdominal pain and the formation of a pancreatic pseudocyst, he was transferred to our institution for evaluation. Dynamic computed tomography (CT) scanning confirmed abnormal vessels in the tail of the pancreas and early venous return to the splenic vein in the early arterial phase. Abdominal angiography revealed a racemose vascular network in the tail of the pancreas, confirming the presence of an arteriovenous malformation (AVM) in this region. This AVM was thought to be the cause of the acute pancreatitis, so a distal pancreatectomy was performed. The patient's postoperative course was uneventful, and there has been no recurrence at the 7-month postoperative follow-up. Surgical resection has a low recurrence rate and good outcome;thus, if a pancreatic AVM appears difficult to treat with conservative medical therapy, surgical resection appears to be the definitive treatment.