Optic disc drusen are acellular calcific deposits occurring in small, crowded optic discs with abnormal vasculature. Evidence suggests axoplasmic transport alteration and axonal degeneration are involved in disc drusen formation. In affected patients, the number and size of disc drusen are highly variable, and the drusen may be visible near the disc surface or buried within the disc, causing them to appear as pseudopapilledema. B-scan echography is the most sensitive method for detecting disc drusen. Most patients with disc drusen are asymptomatic, but progressive visual field loss and vascular complications, including anterior ischemic optic neuropathy and choroidal neovascularization, may occur. Optic disc drusen have no established effective treatment. Diagnosing disc drusen correctly is important to avoid unnecessary work-up and to avoid overlooking potential serious conditions such as true papilledema. Disc drusen patients with more-than-expected visual field defects or progressive visual loss should have work-up to exclude other causes.