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Cystic fibrosis is a systemic autosomal recessive disease manifested by damage to the exocrine glands due to dysfunction of the CFTR gen. The primary defect leads to insufficiency of all exocrine glands with multiple organ manifestations, among which the respiratory system, digestive tract and exocrine parts of the pancreas, as well as the liver, are especially severely affected. Classical basic therapy for cystic fibrosis is symptomatic. Currently, the most modern is pathogenetic targeted therapy using CFTR modulators. The presence of the F508del mutation in a heterozygous or homozygous state is critical for the use of this therapy. CFTR modulators are metabolized in the liver, which makes it necessary to take drugs with caution if it is affected. The article presents a clinical case of the use of fixed targeted therapy with Ivacaftor (75 mg) + Tezacaftor (25 mg) + Elexacaftor (100 mg) and Ivacaftorin (150 mg) a patient with cystic fibrosis complicated by cirrhosis of the liver. The patient’s clinical picture showed progressive multi-organ lesions with a leading clinical problem in the form of liver cirrhosis with signs of portal hypertension and hepatic failure. The presence of the F508del mutation in a heterozygous state made it possible to use fixed targeted combination, but the potential hepatotoxicity of this drug raised concerns. On the other hand, pre-existing liver damage is one of the manifestations of cystic fibrosis, so we can expect at least a slowdown in the progression of liver damage against the background of targeted therapy. By the council decision in June 2022, it was decided to start targeted therapy for vital indications. This case illustrates the success of this triple targeted combination in a child with cystic fibrosis and cirrhosis of the liver, which is a unique observation. Experience in the use of this combination for treatment of cystic fibrosis complicated by cirrhosis of the liver is extremely scarce, therefore, long-term follow-up of the patient is required to assess its efficacy and safety in the long term.