Paragangliomas are very rare tumors arising from neuroectodermal-derived paraganglion tissue. This tumor can be found in any part of the body where healthy paraganglia are known to occur. Malignant pulmonary paraganglioma is distinctly uncommon. We report here a case pathologically proven primary pulmonary paraganglioma with pulmonary metastases and tumor thrombus in a 25-year-old woman. In the patient

non-enhanced pulmonary CT showed a right lung hilar large mass measured 64mm×80mm with a lobulated margin and several nodules close to the pleura. Because of pulmonary metastases and tumor thrombus

the patient had no chance of operation. A bronchial pulmonary artery perfusion chemotherapy and radiotherapy were given. The observation of treatment effect was undergoing. In addition

we review the literature on the topic of pulmonary paraganglioma

management of paragangliomas involving lung.