With complicated pathogenesis and limited treatment options, optic neuropathy is one of the major blinding diseases characterized by damaged retinal ganglion cells(RGC)and axons. Sigma-1 receptor(S1R)is a chaperone protein mainly located in the endoplasmic reticulum membrane, which is abundant in retina and highly expressed in the ganglion cell layer. S1R has been noted increasingly as a novel target for the treatment of neuro-degenerations. More studies have shown that S1R is a pluripotent modulator including Ca²⁺ homeostasis, endoplasmic reticulum stress response, oxidative stress response, neurotrophic factor secretion and glial cell activation, indicating that S1R can generate significant impacts on neuroprotection in neurodegenerative diseases. Additionally, S1R also has neuroprotective effects against RGC loss and dysfunction both in vivo and in vitro, reversing loss partially and maintaining structural integrity, while the absence of S1R exacerbates the disease or increases vulnerability to degenerative diseases. This article intends to review the progress and mechanisms in neuroprotection of S1R in RGC, aiming to provide a new target for the treatment of optic neuropathy.