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Introduction. Persistent left superior vena cava is the most common congenital venous abnormality of the thorax and has a prevalence of 0,3-0,5% among the general population. In most cases, the presence of a persistent left superior vena cava is asymptomatic and is usually a finding during surgery. Most often additional left superior vena cava coexists with superior vena cava in 80-90% cases. This feature is extremely important for choosing the optimal surgical tactics. Case Presentation. Patient Sh., 44 years old, was urgently hospitalized for orthotopic heart transplantation. From the anamnesis it is known that in 2017 the patient was diagnosed congenital heart disease: critical stenosis of the bicuspid aortic valve. However, the patient refused surgery at that moment. Conservative treatment was carried out. In 2022, the patient`s condition progressively worsened: severe exertional dyspnea, increased shortness of breath at rest, pitting edema in the lower extremities, interruptions in the work of the heart. The patient is diagnosed: dilated cardiomyopathy. The patient was included in the waiting list on the heart transplantation. In March 2023, patient underwent orthotopic heart transplantation with reconstruction a persistent left superior vena cava. A tubular conduit from the autopericardium with a diameter of 20 mm and a length of 5 cm was used for reconstruction. An anastomosis was formed between the distal part of the accessory left superior vena cava and the right atrial auricle. The patient was discharged without any complications and no signs of venous congestion in the upper body were detected during the follow-up examination after three months of observation. Conclusions. Our clinical observation demonstrates the feasibility of successful orthotopic heart transplantation using an autologous autopericardium graft in patients with a persistent left superior vena cava and shows encouraging immediate follow-up results.