Congenital epidermolysis bullosa (CEB) is a group of heterogenous, hereditary genetic diseases. Emergence of blisters and erosions on the skin, slight vulnerability of the skin cover, formation of disabling deformities and aggressive skin cancer development are the main clinical manifestations of this pathology. The disease is based on a hereditary genetic defect caused by one or several mutations in the COL7A gene, leading to an insufficient synthesis of type VII collagen functional protein (COL7), which contributes to the occurrence of persistent skin wounds and their infection. Generalized recessive dystrophic epidermolysis bullosa (RDEB) leading to death of patients at a young age is the most severe form of the disease. In the Russian Federation, symptomatic methods aimed at accelerating the healing of erosive and ulcerative skin defects and preventing the development of secondary infection and correcting complications are currently being used to treat patients with CEB, including RDEB. The article presents the first experience of treatment of a patient with RDEB with beremagene geperpavec genetically engineered drug in the Russian Federation. A male patient, 14 years old, with a verified congenital dystrophic epidermolysis bullosa, recessive type, Q81.1 diagnosis was under follow-up. Treatment was carried out at the North-East Clinic with the Children’s Center of the Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology (MSPCDC). Beremagene geperpavec gel was applied to the open wounds located on the skin of the back, right axillary region, inguinal folds, lower extremities. In total, 8 applications were done during 2 months. The treatment had a pronounced therapeutic effect, which manifested itself in complete epithelialization of the wound surfaces.