A 58-year-old man with a complaint of sever epigastralgia was referred to our hospital. At a previous hospital, he had been initially diagnosis as having ruputured aneurysm of the head of the pancreas. A final diagnosis of arteriovenous malformation of the head of the pancreas by MDCT and abdominal angiogram, and underwent pancreatoduodenectomy. Major feeding arteries were the gastroduodenal artery and inferior pancreatoduodenal artery, and the major drainage vein was the gastrocolic trunk. This case was also diagnosed as Osler-Weber-Rendu disease because of the presense of three of the four criteria for this disease: habitual epistaxis, oral telangiectasia, and angiodysplastic lesion in the internal organ. This is the first reported case of arteriovenous malformation with Osler-Weber-Rendu disease in Japan.