Pancreatic arteriovenous malformation (AVM) is a rare disease that occurs worldwide, but only a few sporadic cases have been reported in Taiwan. The poor definition of pancreatic AVM has been the major obstacle in its diagnosis. Conventionally, angiography and CT scans are used to diagnose pancreatic AVM. Here we report a case of pancreatic AVM that was initially disclosed by endoscopic ultrasound (EUS) and confirmed by angiography. In this case report, we will also present the very good response of pancreatic AVM to external beam radiotherapy.