Abstract
INTRODUCTION: Retroperitoneal fibrosis is a fibroinflammatory disease of unknown etiology that can be associated with other fibroinflammatory disorders. Lately, there have been several reports showing that retroperitoneal fibrosis is a manifestation of IgG4-related sclerosing disease, which often presents as autoimmune pancreatitis. CASE REPORT: This report shows the case of IgG4-related sclerosing cholangitis associated with retroperitoneal fibrosis independent of autoimmune pancreatitis. The patient presented with cholestatic jaundice and picture of sclerosing cholangitis in endoscopic retrograde cholangiography. The combination of endoscopic biliary drainage and steroid therapy was an effective treatment in this case. CONCLUSION: The diagnosis of IgG4-related sclerosing disease should always be taken into consideration in patients with sclerosing cholangitis, especially when it is associated with any fibroinflammatory disorder.