Nevus of Ota (NO) also known as nevus fuscoceruleus ophthalmomaxillaris or oculodermal melanocytosis is a rare hamartoma. It is usually characterized by unilateral, patchy and speckled, blue/gray or brown macules on the forehead and periocular area. Here, we report a rare case of NO with oro-buccal involvement, which is an unusual condition. The patient was presented due to the rarity of oro-buccal involvement, extensive and remarkable expression of the lesions, and in order to propose a more comprehensive and neuroanatomy based classification system.