Localized gastrointestinal vasculitis is a rare condition. It may be observed as an incidental unexpected pathologic finding at the time of biopsy of an abdominal mass or may present as unexplained abdominal pain with or without unexplained lower gastrointestinal bleeding. In this report we describe a new case of localized polyarteritis nodosa with involvement of peripancreatic middle-sized blood vessels. A literature review of cases of localized gastrointestinal vasculitis was also conducted. A major point of concern is whether a single organ vasculitis of the gastrointestinal tract is actually a localized gastrointestinal vasculitis or simply an initial manifestation of a more severe systemic vasculitis. Due to this, in cases of localized gastrointestinal vasculitis a complete evaluation of the patient to exclude the presence of a systemic a potentially threatening systemic vasculitis is required.