Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy. Approximately 10% of cases exhibit ocular manifestations leading to serious complications. Specifically, secondary glaucoma can result in severe and blinding eye disease. We present a case of a two-month-old female with JXG related glaucoma in order to demonstrate the classic presentation and the problems commonly encountered in treating this disease. The natural history, pathogenesis, and treatment of the condition are then discussed and the pertinent literature reviewed. JXG should be considered in any case of unilateral glaucoma that presents in infancy.