Sinonasal mucosal melanoma (MM) is a relatively rare and aggressive tumor associated with poor prognosis. MM accounts for 1% of all melanoma, and paranasal sinuses and oral cavity are the most common sites. Although nasal obstruction and epistaxis are the most common symptoms, the development of MM remains insidious. The diagnosis is based on histological and immunohistochemical examination. The 5-year survival rate of MM is around 14%. Surgical resection with negative margins remains the mainstream choice of treatment. Radiotherapy (RT) and chemotherapy are frequently used as adjuvant treatments while no consensus has made these two treatment modalities as standard therapy. In this case, a 54-year-old man was diagnosed with primary sinonasal MM with right parotid gland deep lobe metastasis. He received right endoscopic medial maxillectomy and right parotidectomy after confirming the diagnosis. Adjuvant RT was completed and there is no evidence of recurrence so far at the outpatient clinic. For the rarity of the disease, we presented this case. (J Taiwan Otolaryngol Head Neck Surg 2022; 57:316-320)
