Background: To bring attention to the potential and serious ophthalmic sequalae of Sjogren’s syndrome, specifically occlusive retinal vasculitis. Case: A 70-year-old male patient without previous ocular pathology presented with acute onset, bilateral, painless vision loss. Fundus evaluation revealed extensive retinal occlusive disease with bilateral disc edema, cystoid macular edema, and diffuse phlebitis. The patient was admitted, received a full systemic work-up and was carefully co-managed by ophthalmology, neurology, infectious disease, vascular surgery, dermatology, rheumatology and medicine. Evaluation supported a leading diagnosis of Sjogren’s syndrome associated vasculitis. Conclusion: The patient underwent treatment with oral steroids and intravitreal injections of anti-vascular endothelial growth factor; treatment successfully restored usable vision in one eye. This case will highlight the importance of prompt and thorough evaluation of patients diagnosed with or with suspected diagnosis of Sjogren’s syndrome since severe ocular manifestations can mirror severe, potentially life-threatening, levels of systemic complications.

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