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Purpose: To report the case of a patient who presents with multiple progressive ocular diseases who is diagnosed with concurrent primary Sjögren's syndrome and isolated ocular sarcoidosis. Observation: A 60-year-old woman was referred for dry eye disease, bilateral interstitial keratitis, anterior uveitis, and progressive glaucoma. There was clinical suspicion of an autoimmune etiology due to her ocular history, risk factors, and presentation. Thorough diagnostic testing revealed both primary Sjögren's syndrome and ocular sarcoidosis. After 2.5 years of systemic treatment and follow up, the patient currently remains stable. Conclusions and importance: Autoimmune disease may underlie those with progressive ocular disease with an unknown etiology. More than one autoimmune disease may be the cause of ocular findings, especially for patients with a complicated presentation. Proper awareness, clinical suspicion, and diagnosis of these diseases can greatly improve a patient's condition and prevent future ocular and systemic complications.