Purpose. Demonstration of modern imaging modalities possibilities in the diagnosis and sur-gical correction planning of a rare combined congenital heart disease (CHD) in a newborn. Materials and methods. The article presents a clinical case of patient B., 1 day old, with prenatally suspected complex CHD. Patient was admitted to the intensive care department with signs of decreased saturation. For surgical management of CHD, the patient underwent trans-thoracic echocardiography (TTE) and CT-angiography (CTA) of heart and cardiac angiography. Results. On TTE ductus-dependent CHD, atresia of the pulmonary artery and the absence of interventricular and atrial septa were determined. In the preoperative planning the patient underwent a CTA of the heart and cardiac angiography for a detailed assessment of the pulmonary arteries and patent ductus arteriosus (PDA) anatomy. Identifying possible anomalies of the pulmonary veins and aortopulmonary collaterals was important to determine the surgical tactics. Discussion. TTE has high diagnostic quality in the diagnosis of combined CHD with the pulmonary artery atresia, however, it cannot be considered as a final diagnostic step in surgical correction planning due to limitations in the visualization of extracardiac anomalies. Conclusion. Multimodal diagnostic approach for assessing various anatomical and functional parameters of the heart and extracardiac structures should be used in all children with complex, combined CHD. © 2023 Russian Electronic Journal of Radiology. All rights reserved.