LAUSR

BACKGROUND Nevus of Ota and nevus of Ito are hyperpigmentary dermal melanocytoses which develop as a consequence of disturbances or failures during migration of melanocytes from the neural crest towards the epidermis; they have a relatively unknown aetiopathogenesis and may be congenital or acquired. CASE REPORT This case involves a male patient with a simultaneous diagnosis of nevus of Ota and nevus of Ito at birth. He attended the Neurosurgery department at Carlos Andrade Marin hospital (Quito) with sudden severe headache associated with left brachio-crural hemiparesis. PROGRESS Investigations revealed two extra-axial space-occupying lesions, one parasagittal at the right frontal and parietal lobes and the other located at the right temporal lobe pole. A surgical resection was planned for the parasagittal lesion and the histopathological diagnosis was meningeal melanocytosis. The temporal pole lesion was referred for treatment with Gamma Knife®. CONCLUSION Primary melanocytic neoplasms are extremely rare. There is evidence of their association with dermal melanocytosis and, in particular, with nevus of Ota. This highly unusual case describes the coexistence of two very rare dermal melanocytoses (nevus of Ota and nevus of Ito) and a primary melanocytic neoplasms in the same patient.