Background. The study of tendencies in the incidence and prevalence of hereditary skin diseases, including those manifested by keratinization disorders, is of fundamental importance in practical medicine — epidemiological studies serve as a basis for the organization of medical care. Clarification of the structure of morbidity helps to plan drug provision, diagnosis and optimal health and social care for patients, re-habilitation, sanatorium-resort treatment. Objective. Comparative analysis of the prevalence and incidence of non-syndromic disorders of keratinization among children and adults in Moscow. Material and methods. Evaluation of epidemiological data on the incidence and morbidity of this nosological group was carried out for the 2017—2023 period based on a retrospective analysis of the medical records of patients who underwent outpatient treatment and follow-up at the MSPCDC (Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology). Data from the MSPCDC formed from the information presented in the Form of Federal Statistical Monitoring No. 12 (“Information on the number of diseases recorded in patients living in the service area of a health facility”) were used to study morbidity in the Russian Federation and Moscow as sources of information. Results and conclusion. An analysis of the overall incidence has shown that there is a clear tendency to increase in the number of cases of reported diseases, such as congenital ichthyosis (ICD-10 — Q80) and other specified congenital malformations of skin (Q82.8). The prevalence growth for the 2017—2023 period was 4.76 times and correlates with the increase in the incidence. The incidence of congenital ichthyosis in the Russian Federation has been decreased by 16.0% during the same period and increased by 4.76 times in Moscow. At the same time, the prevalence in the Russian Federation has been increased by 7.15%, in Moscow — by 2.44 times. © 2025, Media Sphera Publishing Group. All rights reserved.