Introduction. Diseases directly related to pregnancy are still leading the maternеal mortality structure, al-though in many countries there is an increase in the number of deaths from extragenital pathology among pregnant women. A significant number of lethal cases in this group are associated with environmental factors, lifestyle, and the overall increase in somatic diseases. The aim of the study was to determine the main causes of maternal mortality and investigate the role of extragenital pathology based on autopsy data. Materials and methods. The study included 29 autopsy observations. We studied medical records, autopsy protocols of deceased pregnant women, women in labor, and puerperant women. Each cause of death was classified according to ICD-10. A clinical and morphological study was also carried out using additional histochemical and immunohistochemical staining methods. Results. The data from pathological studies show that in Moscow, extragenital pathology accounts for up to 40% of maternal deaths. The leading cause of death is circulatory system diseases, including an arteriovenous malformation; a myocardial infarction; a congenital heart disease (patent foramen ovale); cerebral, aortic, and splenic artery aneurysms. The second most common one is other specified diseases and conditions com-plicating pregnancy. This group of diseases included malignant neoplasms. Less common causes of death are gastrointestinal diseases, other diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism that complicate pregnancy, and also respiratory diseases. A single case is associated with a generalized viral infection. Conclusion. Extragenital pathology remains an important obstetric problem, and the management of pregnant women with systemic diseases requires a multidisciplinary treatment approach. Keywords: maternal mortality, extragenital pathology, arteriovenous malformation, cerebral aneurysm, antiphospholipid syndrome