The optic nerve gray crescent can be of clinical significance if unrecognized during assessment for glaucoma. It has a characteristic appearance of a slate gray area of pigmentation within the disc margins and commonly appears along the inferotemporal or temporal neuroretinal rim areas. This type of disc rim pigmentation can create the impression of neuroretinal rim thinning, and thus lead to the misdiagnosis of glaucoma or "glaucoma suspect" with attendant implications for overtreatment or unnecessary close monitoring of such patients. The gray crescent is more common in African Americans than whites (prevalence rate 27% vs 7%) and is bilateral in at least 58% of cases. It has been reported in association with Kjer optic atrophy type 1. Suggested causes of the gray crescent include an accumulation of melanocytes, or retinal pigment epithelium cells partially located in the optic nerve head region if Bruch's membrane extends internal to the peripapillary scleral ring. Other causes of pigmentation that may resemble gray crescent are conus pigmentosus and variations of peripapillary atrophy. When a gray crescent is present, clinicians should endeavour to identify the true anatomical disc margins via the scleral lip and, if necessary, evaluate the patient further with imaging and visual field studies.