Introduction. One of the rare neoplasms of the temporal bone is the dermoid cyst. Data from the world literature contain isolated descriptions of clinical cases of dermoid cysts of the middle ear and suspension fossa, as well as about 20 reports of cases of dermoid cysts localized in the auditory tube. In the domestic literature, only one case of dermoid cyst of the middle ear is described. Purpose. Conduct a brief review of literature data on temporal dermoid cysts and attempt to systematize them. Discussion. The article analyzed the literature data on prevalence, pathogenesis, morphological structure, clinical presentation, diagnosis and approaches to the treatment of dermoid cysts of the middle ear and suspension fossa. Dermoid cyst of the middle ear and fossa is an extremely rare neoplasm, the frequency of which does not exceed 10% in the structure of all dermoid cysts. Said neoplasm is the result of the gastrulation process disturbance at 3–5 weeks of intrauterine development and contains elements of ectodermal and mesodermal origin, which is its distinctive morphological characteristic. Dermoid cyst of the middle ear and suspension fossa can be asymptomatic for a long time. The clinical picture is determined by the peculiarities of the location of the neoplasm, the degree of involvement of the surrounding structures, as well as the presence or absence of extratemporal distribution. The main methods of radial imaging of the dermoid cyst are CT of the temporal bones with intravenous contrast, as well as MRI in T1, T2, DWI modes. Diff erential diagnosis of dermoid cyst of the middle ear is carried out with cholesteatom, epidermoid cyst, cholesterol granuloma. The main treatment method is complete surgical removal of the neoplasm. Conclusion. Despite the development of modern technologies, and approaches to the treatment of ear neoplasms, today there is no algorithm for early diagnosis and planning tactics for the treatment of dermoid cysts of the middle ear and suspension fossa, which often leads to late diagnosis. In addition, according to the world literature, in most of the presented clinical fi ndings of the dermoid cyst of the middle ear and the suspension fossa, it was carried out either intraoperatively or during the histological study of macroreprates. It is necessary to conduct further studies and form a clear algorithm for the early diagnosis of dermoid cysts of the middle ear and suspension fossa to improve the approach to the treatment of this pathology. © 2023, Professionalnye Izdaniya. All rights reserved.