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The role of vitamins in the formation of the immune response, both innate and acquired immunity, is well known. At the same time, deficit of fat-soluble vitamins A, E, D leads to impaired response of the immune system to the infectious invasion and to disorders of immune system functioning. The aim of this article is to analyze the literature data on the impact of fat-soluble vitamins on the function of the human immune system and the possibilities of their use in patients with immunodeficiency. Results . Сurrently, there are enough evidences of the successful use of fat-soluble vitamins in secondary immunodeficiencies. Data on the usage of vitamins A, E, D in the treatment of primary immunodeficiencies are few. However, even reducing of antibiotics and other medicines administration in children with primary immunodeficiency indicates the feasibility of their using. The results of scientific studies on the successful use of vitamins D and A in the treatment of allergic diseases, vitamin D in the prevention and improvement of the treatment of autoimmune and oncological diseases indicate the possibility of their use as adjuvant immunomodulatory therapy in children with primary immunodeficiency. Promising may be the use of vitamins A and E, as powerful antioxidants in patients with primary immunodeficiencies with defects in DNA repair processes. Conclusion . The analysis of the literature data has shown that despite many questions need to be resolved, fat-soluble vitamins A, D, and E, and their analogues can be used in clinical settings to enhance the therapeutic effect in children with immune deficiency., Competing Interests: The authors declare no overt and potential conflict of interest related to the publication of this article., (Copyright© GEOTAR-Media Publishing Group.)