Background: Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple systems. Systemic lupus erythematosus is a disease of 1000 faces and can present with ocular manifestations. Early recognition and treatment are essential to prevent morbidities which can reach up to blindness. Ocular involvement occurs in around 30% of SLE patient. Materials and methods: A case of 40-years-old female complained of an acute periorbital edema with visual involvement concurrent with arthralgia. Results: Ophthalmology evaluation showed ulcerative keratitis. Laboratory investigation showed leukopenia and anemia. Immunological screening showed high positive Anti-nuclear antibody (ANA), anti-double-stranded DNA and low complements level. Magnetic resonance imaging (MRI) of the brain with contrast showed left optic disc swelling and bilateral periorbital edema. The patient was diagnosed as SLE with ocular manifestations and started on pulse steroid therapy with immunoglobulin intravenous infusion. Her ocular manifestation improved and follow up MRI showed improvement of the Left optic disc swelling. Conclusion: Optic nerve disease is a rare ocular manifestation of SLE. It is important to recognize it early to avoid serious complication and blindness.