Primary orbital melanoma in a patient with nevus of Ota

Primary orbitary melanoma is a very rare malignancy which grows adjacent to melanocyte-rich areas. This is the case of a 48 years-old woman with oculodermal melanocytosis who develops a primary orbital melanoma. After the first approach with exeresis and adjuvant radiotherapy and a subsequent management with orbital exenteration and adjuvant immunotherapy; the patient has reached a remission period of 12 months so far. With this case the aims are to emphasize the severe complications potentially associated to the oculodermal melanocytosis and to demonstrate the need of treating complex patients through multidisciplinary teams.