IgG-4 related disease (IgG4-RD) is a recently discovered systemic fibro-inflammatory disease which affects the ocular system. This pathology is not limited only to the orbit, but may also frequently affect the anatomical structures of the eye, as well as other organs. Suspicion of IgG4-RD is based on careful clinical, radiological and immuno-histological examination with a finding of characteristic histopathological changes. Increased values of serum IgG4 need not necessarily be an unequivocal diagnostic criterion for the diagnosis of IgG4-RD. Only a careful histological and immunophenotyping examination together with a clinical finding provide a basis for distinguishing IgG4-RD from other inflammatory pathologies. Corticoids are applied in the treatment of this disease, but they do not exclude the possibility of relapses of clinical manifestations. Second choice pharmaceuticals are azathioprine, mycophenolate mofetil, and the effect of treating relapse of the disease with rituximab is significant.Key words: IgG4 related disease, eye, diagnosis, treatment.