Objective. To conduct a retro- and prospective analysis of the clinical characteristics and results of surgical treatment of patients with congenital temporal bone cholesteatoma. Patients and methods. On the basis of the SPb Scientific Research Institute of ENT of the Ministry of Health of Russia 23 patients were diagnosed with congenital temporal bone cholesteatoma for the period from 2011 to 2018. There were 11 men (47.8%), 12 women (52.2%), the age of patients — from 2 to 44 years. The M.J. Levenson criteria were used to verify congenital middle ear cholesteatoma. (1986). According to intrasurgery findings, we identified congenital cholesteatoma of the middle ear stages according to the ChOLE classification. Other types of congenital temporal bone cholesteatoma included the patients with congenital atresia of the external auditory opening, combined with congenital temporal bone cholesteatoma; we found a patient with exostoses obstructing the external auditory canal combined with congenital cholesteatoma of the middle ear; and a patient with congenital cholesteatoma of the temporal bone pyramid. Results. An increase in the incidence of congenital temporal bone cholesteatoma was noted, which is probably due to increased awareness of specialists and the wider use of imaging research methods. A relationship was revealed between the stage of congenital cholesteatoma according to the ChOLE classification and the necessary volume of surgical intervention. Of the total number of cases of congenital temporal bone cholesteatoma, 26.1% were the patients with anomalies in the development of the outer and middle ear. Conclusions. All patients with suspected congenital temporal bone cholesteatoma require a temporal bone MSCT and middle ear MRI in DWI mode.