Extrinsic pulmonary artery stenosis caused by anterior mediastinum teratoma presenting with an ejection systolic murmur is a rare phenomenon. Till date, 15 cases have been reported (inclusive of this case) in the English literatures. Herein we report a 20 year old female with extrinsic pulmonary artery stenosis because of compression by an anterior mediastinal teratoma with a loud ejection systolic murmur. The case report aims to highlight the awareness of such rare presentation of anterior mediastinal teratomas that may mimic congenital valvular heart diseases among clinicians.
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