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Nevus of Ota, also known as oculodermal melanocytosis or nevus fuscoceruleus ophthalmomaxillaris, is a benign dermal melanocytic nevus that most commonly affects Asian women. While the lesion is largely a cosmetic concern, it has the potential to undergo malignant transformation to melanoma. Patients with nevus of Ota often present to a dermatologist at some point for examination or removal with laser therapy. Therefore, it is important for dermatologists to understand the cutaneous and extracutaneous risks these lesions carry and how they may present to further optimize management and appropriate referrals. This review describes the demographic, clinical, and prognostic features of melanoma in the setting of nevus of Ota, divided by the nature of the primary melanoma: cutaneous, orbital, intracranial.

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