Many people still don’t understand nevus of Ota, but some of them may understand it when they say “black birthmark on the face”. What exactly is a nevus of Ota? Nevus of Ota is usually made up of pinpoint to several millimeters in size spots fused together, a single spot can be round, oval or irregular, and the entire lesion border is irregular and often uneven pigmentation, the entire lesion can be from a few centimeters to the area of unilateral involvement, and occasionally both sides are involved. The color can range from light tan to gray-blue-black, purple-red. Nevus of Ota is often unilaterally affected, most commonly in the first two branches of the trigeminal nerve, and covers the periorbital area, sideburns, forehead, earlobes, anterior and posterior ears, nose, and conjunctiva. Two-thirds of patients will accumulate the ipsilateral sclera, which is a typical feature of nevus of ota, and to a lesser extent, the cornea and iris, fundus, retrobulbar fat, and periosteum. The retina and optic nerve may be involved, as well as iris papillae and glaucoma, but vision is usually unaffected. Other commonly affected areas include the tympanic membrane, nasal mucosa, pharynx, and palate, and the external auditory canal, jaw, or lips, neck, and chest may also be involved. In a small percentage of patients, the lesions are bilateral. The extent of nevus of Ota expands over time and can last for a lifetime, and the color shade can change, especially in relation to cyclical changes in hormone levels, included in menstruation puberty, or menopause. Nevus of Ota is mostly seen in dark-skinned individuals, especially Asians and blacks, as there are reported cases with more females, so it may be misleading to think that the formation of nevus of Ota has something to do with the practical use of cosmetics, but in fact, it is unrelated. The onset of nevus of Ota has two peaks, most of the patients with nevus of Ota appear in infancy within 1 year of age and most of them can appear at birth, and some or half of the patients appear at puberty, and the onset of nevus of Ota at other times is also available but called rare. There are some reports that nevus of ota is familial, but it is generally not considered hereditary. Generally, nevus of Ota is seldom malignant, but the effect of nevus of Ota on aesthetics should not be ignored, because nevus of Ota on the face will cause psychological pressure on the patient because of various external pressures during the patient’s growth process.