We present the case of a 22-year-old woman, who had presented since the age of 15 a pale-blue spot spread on the right-hand side of her forehead and in her bulbar conjunctiva (first and second branches of the trigeminus nerve), consistent with Ota naevus. A few years later another with similar characteristics appeared on the other side of her forehead, cheek and sclera. No deafness, neurological defect nor visual loss were detected. We comment on the rarity of this case because the patient is Caucasian and also we explain the main complications derived of this disease and consider the therapeutic options.