Bilateral nevus of Ota associated with Turner syndrome

In 1939, Ota described the first case of a benign dermal melanocytosis known as nevus of Ota. This condition involves the skin and mucosa innervated by the ophthalmic, maxillary and, rarely, the mandibular division of the trigeminal nerve. It is frequent in people of Japanese descent but is also seen in individuals of Chinese, Indian, African and European descent. Nevus of Ota is non-hereditary pigmentation disorder, which is more frequent in females than males. Unilateral presentation is typically seen, but bilateral involvement is described in 5–10% of patients. We report a 10-year-old Saudi girl with bilateral nevus of Ota associated with Turner syndrome. This is the first report of such an association.