A 69-yr-old man with a past medical history of aortic aneurysm and retroperitoneal fibrosis presented with obstructive jaundice and radiographic features suggesting sclerosing cholangitis and malignancy of the head of the pancreas. A presumptive diagnosis of chronic periaortitis was made, and a dramatic response to corticosteroid therapy was subsequently observed. The clinical course of seven additional patients with pseudotumor of the pancreas associated with retroperitoneal fibrosis is also reviewed; three of those patients also responded favorably to corticosteroid treatment.