Glaucoma is one of the leading causes of blindness worldwide. The current view of glaucoma is that it is a multifactorial disease. Elevated IOP is a recognized etiologic factor which can trigger initial damage through biomechanical and ischemic injury to the retinal ganglion cells. However, elevated intraocular pressure cannot be entirely responsible for the development of glaucoma. Accumulating evidence suggests that abnormal immunity may be contributing to the glaucomatous optic neuropathy. Autoimmunity may be responsible for initiating or exacerbating glaucoma. This review provides an evaluation of the potential role of autoimmunity in some patients with glaucoma.