A 29-year-old white man, with oculodermal melanocytosis, had a rapidly enlarging, erythematous, painful nodule over his left brow, within the nevus, The lesion was excised and diagnosed as a malignant melanoma. Systemic evaluation showed no evidence of distant disease. This is the tenth case reported of a cutaneous melanoma developing in a nevus of Ota. Melanoma arising in the choroid, brain, orbit, iris, ciliary body, or optic nerve in association with a nevus of Ota is well documented. Careful observation is necessary in patients with a nevus of Ota, particularly in white patients, in whom malignant degeneration seems to occur with a disproportionate frequency.