Glaucoma is a chronic neurodegenerative disease characterized by progressive loss of retinal ganglion cells and optic nerve axons, and accompanying loss of visual field sensitivity. With the development of immunology, compelling evidence obtained suggests the involvement of immune system regulation in glaucomatous optic nerve degeneration. Despite the neuroprotective features of immune system, an autoimmune component resulting from a failure to properly control aberrant and stress-induced immune response likely lead to the progression of neurodegeneration in glaucoma. A better understanding of the diverse roles of the immune system in all forms of glaucomatous optic nerve degeneration will facilitate the development of effective neuroprotective strategies in glaucoma. The effect of immune system on glaucomatous neuropathy is reviewed in this article.