The aim of the investigation was to describe a clinical case of the development of interstitial lung lesions in a 57-year-old man with a large left atrial myxoma, which had a reverse development after myxomectomy.Materials and methods. Patient V., 57 years old, was hospitalized in the therapeutic department of the City Clinical Hospital No. 1 named after N. I. Pirogov in connection with bilateral polysegmental pneumonia. A month before hospitalization, he underwent an outpatient examination for progressive dyspnea. Myxoma of the heart was diagnosed. Due to the progression of respiratory failure and the appearance of fever, he was hospitalized. Amidst the multistage antibiotic therapy, there was a torpid course of lung pathology with syndromes of bilateral dissemination and “ground glass”, bilateral lymphadenopathy of the mediastinum, high pulmonary hypertension, and systemic inflammatory reaction syndrome. Thromboembolism of the branches of the pulmonary artery, tuberculosis, sepsis, infective endocarditis, neoplastic processes of pulmonary and other localization were excluded.Results. The clinical picture corresponded to interstitial lung lesions within the framework of paraneoplastic syndrome in heart myxoma. It was decided to urgently carry out myxomectomy.Conclusion. The clinical case demonstrates the development of a rare variant of paraneoplastic syndrome in left atrial myxoma, which was suspected during the patient’s treatment for bilateral polysegmental pneumonia. The progression of the pulmonary lesion was explained by active interstitial inflammation and was supported by the immunological activity of the heart tumor.The performed myxomectomy, despite the persisting syndrome of systemic inflammatory reaction and infiltration of the lung tissue, led in 2 weeks to complete resolution of interstitial lung lesions and pulmonary hypertension, which confirmed the causal relationship between myxoma of the heart and involvement in the pathological process of the lungs.