RESUMEN
BACKGROUND: Melanocytomas are rare pigmented primary lesions of the central nervous system arising from melanocytes of leptomeninges. They occur most frequently in the posterior fossa, Meckel's cave, or along the cervical and thoracic spinal cord. Orbital melanocytomas have been rarely reported. Nevus of Ota is a melanocytic lesion that can be associated with cutaneous and meningeal melanocytic neoplasms. METHODS AND RESULTS: We describe a case of an orbital melanocytoma associated with ipsilateral Nevus of Ota. A 28-year-old man presented with proptosis and an ipsilateral congenital facial melanocytic lesion (Nevus of Ota). After imaging evaluation, a retro-orbital mass was discovered. A needle biopsy was performed and the diagnosis of melanocytoma rendered. The patient underwent complete surgical excision of the lesion. CONCLUSION: In order to make the correct diagnosis and to choose the appropriate therapy, it is important to be aware of this rare presentation and its association with Nevus of Ota.