The article presents an overview of the intermediate results of treatment for maxillary sinus melanoma in a 66-year-old patient. The importance of timely diagnosis and active surgical tactics for achieving the best survival results up to 5 years is noted. After further examination, the diagnosis was confirmed, the patient underwent genomic sequencing of the tumor tissue biopsy, which revealed a BRAF mutation. In this regard, adjuvant therapy was prescribed according to the dabrafenib+trametinib regimen. It was noted that this rare pathology requires careful study, monitoring of clinical outcomes and features of surgical tactics, considering the multifocal nature of the tumor and its high aggressiveness in comparison with the cutaneous form. In the future, the possibility of surgical intervention is discussed, extensive resection is considered, the expected volume, which can reach the removal of both soft tissue and bone structures up to resection of a part of the lower wall of the orbit.