Nevus of Ota and Ito arise from dermal melanocytes and are present at birth in about 50% of cases. A second peak occurs during puberty. The skin lesion is a light to dark brown, blue-black, or bluish-gray macule with a mottled or specked appearance. It increases in intensity and size with time and persists for life. They are more common in Asians (about 75% of cases; most commonly found in the Japanese), in African Americans, and in girls. Nevi of Ota occur on the face, forehead, zygomatic region, periorbital area, cheek, nose, or eye. Ipsilateral eye involvement (ocular melanosis) is commonly seen in moderate to severe cases and may involve the sclera (most common), conjunctiva, cornea, iris, retina, and optic nerve. Other mucous membranes and tissues (ear canal, tympanic membrane, pharynx, hard palate, nasal mucosa, buccal mucosa) may be rarely involved. Nevi of Ito occur on the supraclavicular region, shoulder, upper arm, and neck. Differential diagnosis of these nevi includes postinflammatory hyperpigmentation, café-au-lait spot, or Mongolian spots.