Nevus of Ota is a pigmentation disorder that can be characterized clinically by diffuse bluish-brown staining, more or less discrete, localized on the face, usually on the route of the 1st and 2nd branches of the trigeminal nerve, which is often found in Asians, especially in Japan.

However, over time, cases have been reported among Indians, blacks and exceptionally, the white race. The disease is mainly described among women, usually with an onset at birth or during puberty [1,2].

In this paper we describe the case of a 22 year old Caucasian female, who presented with a diffuse brown-blue coloration located in the zygomatic area, over the lower eyelid and sclera of the right eye that appeared since childhood. The case represents a rarity due to its occurrence in a light-skinned person.