Background: Both iron-overloaded states and the treatment of such states are known causes of optic neuropathy. Observation: A 28-year-old female with history of Hereditary Xerocytosis (HX) with transfusion-dependence on chronic chelation therapy presented with acute, painless, unilateral, sectoral vision loss during her hospitalization for an episode of symptomatic anemia. This is the first reported case of optic neuropathy in a patient with Hereditary Xerocytosis as well as in a patient on deferiprone chelation therapy. Conclusion: Our study highlights the importance of ophthalmologic monitoring in transfusion-dependent patients on chelation therapy, as phlebotomy and prompt chelator discontinuation may potentially arrest retinal damage.