An idiopathic syndrome of acquired hyperopia with choroidal folds has been characterized. Orbital imaging correlates of this syndrome include flattening of the posterior globe and distention of the perioptic subarachnoid space. The mechanism responsible for the clinical and radiographic findings of this syndrome is undefined. Two patients with unusual presentations of papilledema are reported whose clinical and radiographic findings were otherwise identical to those described in the idiopathic syndrome of acquired hyperopia with choroidal folds. One patient had unilateral disc edema and bilateral choroidal folds. The other patient had bilateral choroidal folds observed 2 years before he developed papilledema in both eyes. Both patients had intracranial hypertension, idiopathic in the first, and related to severe chronic obstructive pulmonary disease and cor pulmonale in the second. A third patient is also described who had typical clinical and orbital imaging findings of idiopathic unilateral acquired hyperopia with choroidal folds. He was also found to have mild intracranial hypertension. Intracranial hypertension can cause acquired hyperopia and choroidal folds and may be the underlying mechanism in some patients with what appears to be idiopathic acquired hyperopia with choroidal folds.