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Background: Graves' orbitopathy is an inflammatory orbital disease that represents the commonest extrathyroidal manifestation of Graves' disease. Autoimmune pancreatitis (AIP) is a rare inflammatory disease characterized by prominent lymphocytic infiltration and fibrosis of the pancreas causing organ dysfunction. Summary: This report provides the first clinical description of severe Graves'-like orbitopathy occurring in association with AIP. Although there was no clear evidence of autoimmune thyroid disease or dysfunction in our patient, the clinical course of his orbitopathy was related to that of AIP, the relapses of orbital inflammation being temporally coincident. Conclusions: Our data suggest that shared autoantigens between the pancreas and the orbit might be responsible for the unusual disorder observed in our patient. [ABSTRACT FROM AUTHOR]