Primary mucosal melanoma of the oral cavity is an exceedingly rare neoplasm 
which is estimated to comprise 1-2% of all oral malignancies. In contrast to 
cutaneous melanomas, the risk factors and pathogenesis are poorly understood. 
The predominate localization of primary oral melanoma is hard palate and 
maxillary alveolus. Dermoscopy may be utilized as an adjunctive tool in the 
clinical differential diagnosis of oral mucosal melanoma whenever the lesion is 
accessible with a dermoscope. Surgery is the mainstay of treatment, but it may 
be challenging depending on the location of the tumor within the oral cavity and 
its size. Adjuvant therapy with dacarbazine, platinum analogs, nitrosoureas and 
interleukin-2 have been utilized with low response rates. Imatinib may be 
effective for patients with with c-Kit gene mutations. Sunitinib and dasatinib 
have been reported effective in selected cases. Vemurafenib and dabrafenib are 
targeted agents for patients with BRAF mutation-positive melanoma. Ipilimumab, 
an anti-cytotoxic T-lymphocyte antigen 4 antibody and pembrolizumab, a 
monoclonal antibody targeting programmed death 1 receptor may be a feasible 
treatment option in patients with metastatic mucosal melanoma.