Melanocytoma is a benign, congenital, highly pigmented tumor with poorly defined borders that typically appears on or adjacent to the optic nerve head. It usually remains stable, although 10% may experience slow growth. It must be differentiated from other lesions such as melanoma and choroidal nevus.
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A 37-year-old asymptomatic woman presented with a unilateral pigmented lesion on the left optic disc. Multimodal imaging confirmed the diagnosis of malanocytoma associated with a choroidal nevus. This lesion has remained stable with a 10-year follow-up.
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