IgG4 related disease (IgG4-RD) is a distinct entity that frequently occures in an ophthalmic location. IgG4 – RD is not limited to the orbit but may also involve other anatomical structures in and around the eye. A high level of suspicion for the diagnosis can be derived from careful clinico-radiologic examination, the use of immunohistological examination in the context of characteristic histopathologic features. Serum IgG4 levels are neither sensitive nor specific for the diagnosis of IgG4-RD and should not relieved upon solely. Careful evaluation of histologic and immunophenotipic features and clinical correlation are required to distinguish orbital IgG4-RD from other inflammatory lesions in the orbit. Glucocorticoids are the first-line drugs for therapy of IgG4-RD. Azathioprine or mycofenolat mofetil can be used as a second possibility. Rituximab can be effective in the patients with relapse IgG4-RD.