Total blindness from presumed optic nerve melanocytoma

PURPOSE: To describe total blindness secondary to presumed optic nerve melanocytoma. DESIGN: Interventional case report. METHODS: A 12-year-old African-American girl, who developed loss of light perception secondary to a pigmented lesion of the optic disk, underwent transvitreal fine-needle aspiration biopsy (FNAB) of the lesion. RESULTS: Cytopathology revealed cells with features of melanocytoma and melanophages but no cells compatible with melanoma. The patient is being followed without treatment. CONCLUSIONS: Optic nerve melanocytoma can cause total blindness in the affected eye. Although it has limitations, FNAB can assist in diagnosis and management of melanocytoma with visual loss.