The present report describes a 40-year old female in whom CT showed tumor of the right lung with bronchus compression. VTS showed tumor of the right lung with pericardium involvement but VTS biopsy could not identify the type of tumor. The patient underwent right pneumonectomy with pericardium resection. The diagnosis of a rare lung disease – primary leiomyoma of lower respiratory tract was confirmed only by immunohistochemistry.

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