Purpose: The purpose of this report is to describe a clinicopathologic correlation of an unusual pigmented tumor of the optic nerve head, to point out that such a lesion can simulate clinically a melanocytoma of the optic disc, and to discuss the differential diagnosis of pigmented epipapillary lesions.
Findings: Histopathologic studies of the affected eye showed a peculiar pigmented tumor of the optic disc that was compatible with an adenoma arising from the juxtapapillary retinal pigment epithelium.
Conclusions: Adenoma of the retinal pigment epithelium can closely simulate a melanocytoma of the optic disc. There are some clinical features that may serve to differentiate the two lesions.