| Summary: | Samson Dires Gashaw, Waltengus Demissie Birhanu, Fitsum Alemayehu Department of Otolaryngology, Head and Neck Surgery, St. Paul’s Hospital Millennium Medical College, Addis Ababa, EthiopiaCorrespondence: Waltengus Demissie Birhanu, Email waltebirh@gmail.comBackground: Intranasal mucosal melanoma is a rare form of melanoma. Presenting as the features of occult malignancy and rapid in its progression. Presented with nonspecific symptoms. So far, no specific risk factor has been identified. The histopathological and immunohistochemical examination helps to confirm the diagnosis. Here, we present a case of primary intranasal melanotic mucosal melanoma and literature review.Case Report: The authors present a patient with primary right intranasal melanotic mucosal melanoma.Conclusion: An endoscopic medial maxillectomy was done, and the patient was linked to the oncology department for radiotherapy.Keywords: mucosal melanoma, ectodermal, endoscopic medial maxillectomy
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