Forty children with pseudopapilledema due to optic disc drusen (31 bilateral, nine unilateral) were studied retrospectively to characterize the early natural history of this condition. The average age at the first examination was 10.2 years (range 3.6 to 19.5 years), and the mean follow-up period was 44 months. Discrete hyaline bodies or papillary calcifications were first noted in one or both eyes with pseudopapilledema at a mean age of 12.1 years in 38 of 40 cases. Decreased visual acuity due to drusen occurred in only one eye due to subretinal neovascularization. Visual fields obtained in 35 eyes (21 patients) with pseudopapilledema showed an enlarged blind spot (9), an inferior arcuate/sector/or altitudinal defect (6), or both (3) in 18 eyes. Our study suggests that discrete papillary calcifications or hyaline bodies frequently emerge and visual field deficits are commonly detectable in the second decade of life in patients with pseudopapilledema due to optic disc drusen.