Melanocytoma in the orbital apex

We report a case of a 49-year-old man presenting with a melanocytoma located in the orbital apex. The tumor was subtotally removed and adjuvant chemotherapy was given. The patient has remained under follow-up for the past 12 years without any evidence of recurrence. Although the characteristic neuroradiological images of the lesion prove the presence of melanin, histological examination is crucial to establish the diagnosis of melanocytoma. Besides the clinical and neuroradiological presentation, the histological, immunohistochemical, and ultrastructural findings are described. To our knowledge, this is the second published case of intraorbital melanocytoma initially confined to the orbital apex. Melanocytoma should be included in the differential diagnosis of intraorbital tumors in such a location.