Optic disk drusen occur in 3.4 to 24 per 1,000 population and are bilateral in approximately 75%. Disturbance in the axonal metabolism in the presence of a small scleral canal--regardless of eyelength--is considered responsible for the development. The drusen increase in size, becoming more visible with age due to continuing calcium apposition, and they are associated with visual field defects in a considerable number of patients. Patients do not usually notice these defects, despite their progressive nature over the years, and this indicates an insidious course. A correct diagnosis of optic disk drusen is mandatory, although effective treatment is not yet available. It is most important to differentiate optic disk drusen from papilledema in order to avoid unnecessary neurological examinations, but also to avoid overlooking genuine neurologic disorders. Because optic disk drusen can cause severe visual field defects, patients require individual consultation regarding work issues and whether or not to drive. Optic disk drusen can be accompanied by vascular complications as well. In some cases these vascular changes--for example, choroidal neovascularization--are treatable. Patients with optic disk drusen should undergo regular visual field, IOP, and nerve fiber layer examinations. In patients with deteriorating visual field and borderline IOP, we recommend antiglaucomatous therapy.