Purpose: To report a case of recurrent nummular keratitis in a pediatric patient with Hyperimmunoglobulinemia D syndrome. Methods: A retrospective chart review. Results: A 14-year-old boy with Hyperimmunoglobulinemia D syndrome (HIDS) presented with photophobia and ocular irritation concomitant with disease exacerbation. He was found on exam to have significant nummular keratitis, which responded to a short course of topical steroids. Despite acute response to local immunosuppression, the patient had several recurrent attacks and eventually developed a large corneal scar and decreased vision. After initiation of infliximab therapy his ocular sequelae improved dramatically and his vision returned to 20/20. Conclusion: One possible form of end-organ damage associated with HIDS is vision threatening nummular keratopathy.