Bilateral naevus of Ota with choroidal melanoma and diffuse retinal pigmentation in a dark skinned person.

Naevus of Ota (naevus fusculocoeruleus ophthalmomaxillaris) was described by the Japanese dermatologist, Ota, in 1939 as a dermal melanocytic hamartoma that presents as bluish hyperpigmentation along the ophthalmic, maxillary, and mandibular branches of the trigeminal nerve. It is bilateral in less than 5% cases, occurring frequently in Orientals (0.2%–1%) and darker races and rarely in white people (0.04%). Open angle glaucomas and choroidal melanoma are the rare ocular involvements. Ota’s naevus is more common in Asians than white people but uveal melanoma occurs predominantly in white populations. 2 Dark skinned patients represent only 1% of all cases of orbital melanomas. The risk of developing uveal melanoma in a patient with naevus of Ota is one in 400 patients in their lifetime. 2 We report a rare case of bilateral naevus of Ota with a right (RE) choroidal melanoma and left (LE) diffuse pigmentation of retina.