Arteriovenous malformation (AVM) of the pancreas is exceedingly rare, although it may be increasingly diagnosed due to the widespread use of cross-sectional imaging of the abdomen; even rarer is its association with pancreatitis. A 45-year-old male was admitted to our hospital with symptom of epigastric pain. Dynamic contrast imaging scans of the abdomen showed a focal acute pancreatitis and pancreatic AVM. The angiography findings were compatible with pancreatic AVM. The cause of abdominal pain was diagnosed by acute pancreatitis caused by pancreatic AVM, and, for definitive treatment of symptomatic pancreatic AVM, distal pancreatectomy with splenectomy was performed. The histological results confirmed the presence of irregular dilated tortuous arteries and veins, and a retention cyst with epithelial cell lining. We experienced a rare case of a 45-year-old-Korean male who had acute pancreatitis associated with pancreatic AVM. Pancreatic AVM is extremely rare; even rarer is its association with pancreatitis.