Background. In connection with the emergence of new methods of treatment of primary biliary cholangitis, it has become important to determine the number of these patients, as well as the main epidemiological indicators of this disease in Russia. Objective: to determine the main epidemiological indicators of primary biliary cholangitis in Russia. Methods. A response was received from the constituent entities of the Russian Federation to the request on the number of patients with primary biliary cholangitis in children 0–14 years old (13 regions), 15–17 years old (7 regions) and adults (51 regions). An algorithm for excluding potentially unreliable values was developed and applied. Estimated prevalence of primary biliary cholangitis was calculated as well, as the number of patients. The estimated incidence of primary biliary cholangitis was also calculated. Results. The estimated prevalence of primary biliary cholangitis in children 0–14 years of age is in the range of 0.20 to 0.98 (conservative estimate 0.24–0.65) per 100,000 children 0–14 years of age; the estimated prevalence in children 15–17 years of age is in the range of 0.08 to 4.46 (conservative estimate 0.31–2.69) per 100,000 children aged 15–17 years. The estimated prevalence in adults is in the range of 2.41 to 5.21 per 100,000 adults (conservative estimate 4.37–5.19). The estimated prevalence of primary biliary cholangitis of the total population in the Russian Federation ranges from 1.95 to 5.21 (conservative estimate 3.53–4.33) per 100,000 population. The estimated incidence of primary biliary cholangitis in Russia is most likely in the range of 0.11 to 0.24 per 100,000 population. Conclusion. The estimates obtained suggest that there are 2857 to 6525 (conservative estimate from 5159 to 6332) patients with primary biliary cholangitis in Russia. The prevalence score currently corresponds to diseases with orphan status.