A 59-year-old patient presented with metastatic uveal melanoma that develop
ed in a nevus of Ota. The nevus of Ota or oculodermal melanocytosis contain
s an increased number of dermal melanocytes in the distribution of the opht
halmic and maxillary divisions of the trigeminal nerve. Malignant transform
ation arising in a nevus of Ota may occur in all anatomical sites influence
d by the nevus. Most often the choroid is involved. Although the nevus of O
ta 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.