The present study reports the case of a 79-year-old woman manifesting skin changes, a pancreatic neoplasm, enlarged lymph nodes, an eyelid mass and interstitial pneumonia over a 30-year period. At 2 months before admission to our hospital, the patient presented rapid vision loss in the left eye. Left optic nerve atrophy with a focal hyperintense lesion was documented on a T2-weighted magnetic resonance imaging (MRI) scan, and visual evoked potential implicit times were prolonged. Elevated serum immunoglobulin-G4(IgG4) concentrations and the presence of enriched IgG4-positive plasma cells in the lymph nodes established the diagnosis of IgG4-related optic neuropathy. Following oral treatment with methylprednisolone, the serum IgG4 levels fell to normal levels, and the left eye visual acuity improved to a level that remained stable over a 1-year follow-up period. After 4 months of methylprednisolone administration, the optic nerve appeared to be normal on an MRI scan. Prior reports on IgG4-related optic neuropathy involved an infiltrating mass, which was not observed in the present case. Similarly to the current study, previous cases have responded to treatment with glucocorticoids, indicating that the underlying mechanism of the disease may be common.