Pancreatic arteriovenous malformation is a rare entity (0.9%). There are about 200 articles available in PubMed. This review article includes 86 published articles, with 117 cases published after 2000. The median age at diagnosis was 51, and most of the patients were male (87.0%). The symptoms included pain, bleeding, pancreatitis, ulcers in the duodenum or stomach, varix formation, jaundice, and ascites. The diagnostic modalities were angiography, contrastenhanced CT, MRI, and/or Ultra Sound. The most common treatments were surgery and embolization. The clinical success rate of embolization reported was 57.7%. The tailored embolization based on each agio-architecture had a clinical success rate of 80%. If embolic therapy is ineffective, surgical intervention should be considered.