Mucosal melanoma of the nasal cavity and paranasal sinuses is a rare disease, but its incidence appears to be increasing. The mean age at diagnosis is between 65 and 70years. Unilateral nasal obstruction and epistaxis are the most common presenting complaints. Melanoma arises in the septum or lateral wall of the nasal cavity in the great majority of cases. The histological diagnosis is based on specific immunohistochemical labelling and is usually established at an advanced stage of disease: stage T3 or T4 tumours according to the 7th edition of the American Joint Committee on Cancer (AJCC) classification of tumours. First-line treatment consists of surgery. The place of intranasal endoscopic surgery remains controversial due to the difficulty of controlling surgical margins and should be reserved for experienced teams. Adjuvant radiotherapy is usually performed due to its efficacy on local and regional disease control. Five-year overall survival of mucosal melanoma of the nasal cavity and paranasal sinuses in the most recent series does not exceed 40%. Local recurrence is observed in about 50% of cases and metastatic disease is common. The quality of initial tumour resection with negative surgical margins is the most important prognostic factor for tumours confined to the nasal cavity. Hopes for improvement of survival are based on early diagnosis, progress in radiotherapy techniques and cell and gene therapy that are currently under evaluation.