Arteriovenous malformation of the pancreas is a rare disease, and it is manifested by gastrointestinal bleeding and/or portal hypertension. Surgery is definitely the treatment of choice at the early stage of the disease, and a transcatheter embolization is an alternative treatment for the control of bleeding and if the lesion is surgically inaccessible. We describe a 62-year-old man who had refractory ascites and esophageal variceal bleeding caused by a pancreatic arteriovenous malformation associated with portal hypertension; this was successfully treated by a transjugular intrahepatic portosytemic shunt.