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We describe a case of antineutrophil cytoplasmic antibody (ANCA)-associated retinal vasculitis in a 21-year-old previously healthy Malay woman, who presented to us with complaints of sudden painless loss of vision in her left eye. Her vision upon presentation was counting fingers and her fundus examination showed retinal vasculitis and ischemic changes. Fundus fluorescein angiography showed leakage from the vasculitic retinal vessels, resulting in macular oedema. All investigations were normal, except that a full blood test showed eosinophilia and an autoimmune screening revealed positive perinuclear staining-ANCA, which led us to diagnose ANCA-associated retinal vasculitis. The patient was comanaged with the rheumatology team. She was started on a high-dose intravenous steroid followed by a tapering dose of an oral steroid. Improvement was noted from the resolution of macular oedema evident on optical coherence tomography of the macula. However, vision remained poor and unchanged.