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Purpose of the study. Improvement of the results of treatment of patients with arteriovenous forms (AVF) of congenital vascular malformations (CVM) of the extremities based on the development of the classification scheme of CVM and the study of proliferative activity of the pathology. Materials and methods. The clinical data of 155 patients with AVF of CVM of extremities were analyzed in terms of observation from 1 month to 10 years. Patients of sex of men there were 65 (42%), women – 90 (58%), their correlation – 1 : 1,4; the average age was about 25,1 ± 10,4, children's age group – 53 (34%). The division into clinicalanatomical forms (11 groups) was carried out on the basis of the «working» classification scheme «VASC + T». Expression proliferation markers VEGF and KI-67 woos study were performed by immunoperoxidase method with additional hematoxylin staining. Results and discussion. Pathomorphological (66; 45,8%) and immunohistochemical studies (10; 7%) revealed the presence of proliferative activity of angiomatous tissues and degenerative changes in the walls of the vessels both due to developmental defects and disorders of hemodynamics, while the source of progression of the AVF of CVM there are vessels of the microvasculature. Conclusions. The application of the CVM classification scheme and the step-by-step complex treatment approach, depending on the clinical and anatomical AVF of CVM, led to the absence of major postoperative complications. This tactic made it possible to achieve satisfactory long-term results in 136 (94,4%) cases.