RESUMO
Malignant melanoma is an aggressive malignancy of melanocytes which is usually found on sun exposed areas of the body. A rare variant of this disease with no etiological association is the mucosal malignant melanoma found on all mucosal surfaces of the body including the oral cavity, respiratory mucosa and anorectal region. In the head and neck region, this disease is almost always diagnosed at an advanced stage and requires a very high index of suspicion for diagnosis. It is more commonly found in females than males.Indians are more prone to this disease as compared to Caucasians.Due to the obscure location within the oral and nasal cavity, it is clinically found at an advanced stage and requires surgical resection with adequate margins for complete eradication. This may be achieved either endoscopically in the nasal cavity or with wide local resection in the oral cavity. this in certain cases may not be feasible due to vicinity of vital structures. In such cases, adjuvant radiotherapy helps in the local control of disease. Histopathological evaluation of the specimen helps to determine aggressive biology of tumor with factors such as presence of ulceration, nodular morphology and perineural invasion being high risk features for development of local and regional recurrence. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04001-y.