Introduction Acquired pulmonary artery stenosis is a rare entity that has scarcely been described in the literature, and it is mainly associated with compression of the pulmonary artery trunk and its branches by mediastinal tumors (teratomas and lymphomas). We report the case of a young patient with non-Hodgkin lymphoma in the anterior mediastinum that led to extrinsic compression and local luminal invasion of the pulmonary artery trunk, causing significant impact on the right heart chambers, with subsequent improvement after chemotherapy. […]