Purpose: To describe a case of granulomatous anterior uveitis and histologically confirmed chronic granulomatous conjunctivitis in the presence of common variable immune deficiency (CVID). Methods: Interventional case report. Results: A 72-year-old female with a history of CVID treated with regular intravenous immunoglobulin (IVIG) infusions developed chronic conjunctivitis and granulomatous anterior uveitis. She responded to topical steroids, but there was recurrence upon cessation of steroid therapy. Conjunctival biopsy demonstrated micro-granulomas in the stroma and epithelium. Treatment with IVIG was maintained throughout. Conclusion: Although rare, a diagnosis of CVID should be considered in patients with recurrent conjunctivitis and uveitis of unknown etiology, especially if there is a clinical history suggestive of defective immunity. They tend to respond well to continued steroid therapy, and IVIG therapy should not be stopped. [ABSTRACT FROM AUTHOR]