Highlights. The paper presents a unique clinical case of patient with congenital heart defect known as Bland-White-Garland syndrome diagnosed in the adulthood. Takeuchi repair (creation an intrapulmonary tunnel) was carried out to treat this defect.Background. An anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome) is an uncommon but severe pathology that requires immediate surgical treatment. The use of modern diagnostic methods (both non-invasive and invasive) has resulted in higher prevalence of this syndrome, diagnosed not only in an early childhood, but also in adulthood. Recording each medical case and choosing an optimal treatment strategy will increase knowledge of this pathology and reduce the high risk of mortality. The paper presents a clinical case of this syndrome in an adult patient who had remained undiagnosed for a long time as the syndrome was masked under the clinical features of chronic heart failure. Takeuchi repair (creation an intrapulmonary tunnel) was carried out to treat this extremely rare defect for a patient of this age group.