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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.1,2 Dark skinned patients represent only 1% of all cases of orbital melanomas.3 The risk of developing uveal melanoma in a patient with naevus of Ota is one in 400 patients in their lifetime.1,2 We report a rare case of bilateral naevus of Ota with a right (RE) …