Primary malignant melanoma (MM) arising from the parotid gland is rare, and only sporadic cases have been published. MM was usually presented as metastases from cutaneous or mucous sites of the head and neck regions. This entity is characterized by delayed diagnosis, controversial pathogenesis, and poor prognosis. Treatment options are limited particularly for advanced lesions. Herein, a case of parotid gland malignant melanoma in an 86-year-old man with no other primary lesion with detectable and distant metastases in a short interval was reported. Definitive diagnosis made by core needle biopsy of the parotid mass was confirmed by immunochemical analysis, revealing positive staining for S-100 protein, HMB-45, and Melan-A. Whole-body PET/CT image revealed distant metastasis, including the bone marrow of the whole skeleton, such as the sternum and spine. Thereafter, the patient was managed with hospice care by family’s requests because of old age and life-quality expectation. This case highlights the importance of making definitive diagnosis, pathological feasibility, and treatments with a review of the literature. (J Taiwan Otolaryngol Head Neck Surg 2023; 58:116-121)
