Abstract
Nevus fusco-caeruleus ophthalmo-maxil-laris Ota is a pigmentary disorder confined to the eyelids, zygomatic and and frontal regions, and sometimes to the nose, ear lobe, retroauricular region and the anterior portion of the scalp. A first case of this nevus with pigmentation on the lips is reported by us. In this case, pigmentary anomaly of the face is bilateral, and associated with pigmentation of the lateral cervical area, upper thorax, and the right shoulder (nevus fusco-caeruleus acromiodeltoideus Ito).