We postulated that an alteration in endogenous vitamin A (retinol) metabolism plays a causal role in the pathogenesis of idiopathic intracranial hypertension (IIH).Serum retinol was determined by a fluorometric method from 40 control subjects and 58 patients with idiopathic intracranial hypertension (IIH). Retinol binding protein (RBP) was also assayed by quantitative radial immunodiffusion in 17 control subjects and 30 patients with IIH.Mean retinol values were higher in the IIH group compared with the control group, but did not reach a significant level. However, seven of 30 patients with IIH had high RBP levels, but none of the control subjects did.This data suggests that IIH is associated with an abnormality in vitamin A metabolism that is linked to its transport system.