A 46-year-old man presented with acute epigastric pain and was diagnosed with acute pancreatitis. Although successfully managed, it recurred shortly after; he was subsequently referred to our institute for further investigation. Dynamic computed tomography (CT) and endoscopic ultrasonography revealed an arteriovenous malformation (AVM) in the body and tail of the pancreas. A diagnosis of pancreatic AVM causing recurrent pancreatitis was made and distal pancreatectomy was performed. Follow-up CT after surgery confirmed no further recurrence of either the AVM or pancreatitis. AVM of the pancreas is rare, and surgical resection is necessary in symptomatic patients.