AIM: This study reports a case of malignant melanoma localized primarily to the nasal cavity in the context of the relevant literature. CASE REPORT: A 43-year-old man presented complaining of nasal obstruction that had worsened progressively over the previous 8 months. The obstruction localized predominantly to the right side, and was associated with recurrent self-limiting epistaxis and weight loss (10 kg in 3 months). The physical examination revealed a large, pale red tumor within the right nasal cavity that was pushing the nasal septum towards the left nasal cavity. There were no identifiable cervical lymph nodes, changes in the oral cavity or otoscopy. A sinonasal computed tomography scan revealed hypodense material occupying the nasal cavity and paranasal sinuses to the right, extending towards the rhinopharynx, without signs of bone destruction or erosion. The anatomopathological analysis revealed a poorly differentiated malignant neoplasm. The immunohistochemical results were consistent with malignant melanoma. Conclusions: The presentation of a melanoma in the primary mucosa is rare, accounting for approximately 2% of all melanomas. Melanomas are tumors originating from melanocytes, cells derived from the neuroectoderm located in the basal layer of the skin, its attachments, and more rarely in the mucosa. Mucosal melanomas typically involve the nasal cavity, and these account for 4% of all sinonasal malignancies. This case report, which describes a rare tumor of the nasal cavity, is expected to improve the recognition and referral of this condition by ear, nose, and throat (ENT) specialists for multidisciplinary management.