Aim: to present an exceptional case of the combined pathology – an abnormal drainage of portal system to the coronary sinus, hypoplasia of the right branch of the portal vein and high pulmonary hypertension. Materials and methods. The patient P. of 22 years old arrived at congenital heart diseases surgical unit of V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs. After the examination he was diagnosed with congenital disease – abnormal confl uence of the portal vein in the right atrium. Insuffi ciency of the tricuspid valve 3–4. High pulmonary hypertension. Insuffi ciency of blood circulation of IIa, III functional class. After computer tomography there is no division of the portal vein. Vein is drained in the right atrium bypass with a diameter of 2,3 cm following from the portal vein to the coronary sinus. Results. Considering hopelessness of conservative therapy the only method of the radical help to the patient is two-stage surgical intervention – transplantation of a heart-pulmonary complex and liver transplantation. Conclusion. Presented rare clinical case demonstrates the combination of congenital anomalies of the portal system and the heart with high pulmonary hypertension, and is based on a functioning fetal venous duct (ductus venosus), bypassing the liver in the right atrium (coronary sinus) in combination with hypoplasia of the right portal vein.