Purpose: Optic disc drusen (ODD) is hyaline deposits in the optic nerve head seen in 1-2% of the population. Long-term evolution of ODD anatomy and visual field defects in ODD patients is a key factor for learning more about pathophysiology and prognosis of the condition. With a median follow-up period of 56 years, this is the first study that evaluates superficial optic disc anatomy and visual fields in patients with ODD over a life span. Methods: Observational case series investigating progression of superficial optic disc anatomy and visual fields in ODD patients. Eight patients with hereditary ODD were included. Results: Mean age at initial and follow-up examination was, respectively, 16.8 and 73.3 years. The median follow-up time was 56 years. A minimal or non-existing change in superficial ODD anatomy (size and number) was seen in 10 of 12 eyes. There was a tendency towards more anatomical change in subjects younger at first examination. A 27% decrease in Goldmann perimetric visual field area (cm²) was found between initial and follow-up examinations. Conclusion: Minimal or no change in superficial optic disc anatomy and visual fields in ODD patients oldest at the initial examination suggests that anatomical progression of ODD in patients with hereditary ODD happens primarily before adulthood.