Purpose: To report the association of optic disk melanocytoma with systemic hypertension. Cases: Ocular examination of 3 asymptomatic hypertensive patients revealed a pigmented lesion over the optic disk. After clinical examination and radiological evaluation a diagnosis of optic disk melanocytoma was made. One patient had a mild decrease in visual acuity associated with an increase in size of the lesion during the follow-up. Systemic evaluation revealed increased urinary vanillylmandelic acid in all the patients. MRI showed diffuse adrenal hyperplasia while a 131I-MIBG scan showed increased uptake in 1 case. Conclusions: Optic disk melanocytoma is a benign tumor though the lesion may increase in size with the resultant decrease in visual acuity over few years. It may be associated with systemic hypertension secondary to increased levels of catecholamines in the body, which is related to the common neural crest origin of melanocytes, adrenal medullary cells and chromaffin cells.

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