A 59-year-old patient presented with metastatic uveal melanoma that developed in a nevus of Ota. The nevus of Ota or oculodermal melanocytosis contains an increased number of dermal melanocytes in the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve. Malignant transformation arising in a nevus of Ota may occur in all anatomical sites influenced by the nevus. Most often the choroid is involved. Although the nevus of Ota is rare in Caucasians, associated melanoma is more common than in Asians or black people. The dermatologist should be aware of this problem and aim at an interdisciplinary management of these patients.