A 69-year-old man presented with obstructive jaundice and dark urine. Contrast-enhanced computed tomography revealed an enlarged pancreas with homogenous enhancement. Endoscopic retrograde pancreatography demonstrated short-segmental, irregular narrowing of the main pancreatic duct. The patient underwent exploratory laparotomy and needle biopsies of the pancreas, which showed marked fibrotic change with lymphocyte infiltration. These clinicopathologic findings suggested autoimmune pancreatitis. Four years later, computed tomography demonstrated marked periaortic soft tissue surrounding a calcified infrarenal abdominal aorta compatible with retroperitoneal fibrosis. We diagnosed retroperitoneal fibrosis with noncontiguous pancreatic fibrosis. This patient responded well to corticosteroid treatment. Autoimmune pancreatitis associated with idiopathic retroperitoneal fibrosis seems to be extremely rare, and to our knowledge, only a few cases have been reported.