A 67-year-old woman experienced acute unilateral visual loss accompanied by pain with eye movements. There was a marked relative afferent pupillary defect and a nerve fiber bundle defect in the upper half of the visual field. Optic discs were normal. After 4 days vision worsened to motion detection and only a temporal island was left in the visual field. The optic disc margin was blurred. Since thirty years she had been suffering from renal insufficiency. Immunoserologic examination revealed elevated ANA and DS-DNA antibody titers. An optic neuritis in systemic lupus erythematosus was diagnosed, which is called atopic, because of its association to a systemic disease and the old age of the patient.The patient was treated with 100 mg prednisolone/day, slowly tapered. Within 6 weeks visual acuity improved to 0.6 and visual field normalized except for a small nerve fiber bundle defect.Autoimmune optic neuritis often responds to treatment with corticosteroids. Early onset of treatment is important. Immunopathologic examinations are an important diagnostic tool in atopic optic neuritis. Their results may even have consequences for the treatment of the underlying disease.