Primary sinonasal mucosal melanoma (SNMM) is an aggressive malignant tumor with high metastasis and poor prognosis. The symptoms of SNMM are non-specific, with the nasal cavity being the most common site of origin. SNMM is diverse in histopathology. The profiling of SNMM gene expression has identified a number of common genetic mutations. These mutations may provide potential targets for emerging biologic therapies. In addition, understanding the staging of TNM is of great clinical significance. The combination of multimodal therapy on the basis of surgery may benefit patients. Patients with SNMM are often in advanced stages at the time of presentation. In this paper, we summarized the latest information of SNMM in recent years, including its pathogenesis, histopathological features, specific molecular markers, clinical stage, clinical treatment plan and prognosis.