The clinic data，imaging findings and pathological features of a PGL patient was collected， the clinical manifestations，imaging fingdings and pathological feaures were analyzed，and the relevant literatures of PGL in recent years were reviewed；the standard diagnosis and treatment criterion of PGL was summarized.

A 57 years old female patient was hospitalized because of left lung nodules for 10 d found in medical examination.The chest CT of the patient received 10 d before showed round-like shape density shadow of soft tissue of up left lung and low left lung，so the patient went to our hosptal for furth diaganosis and treatment. The patient underwent glomus jugulare tumor resection surgery in 2018 in other hospital. The PET-CT results after hospitaliztion showed that the nodule in the low left lung was considered as malignancy， the metabolism of the nodule in up left lung was not high，and malignant lesion was not excluded.The paitient received wedge resection of up left lung and low lef lung nodules assisted with thoracoscope and lymph node sampling；the results of rapid pathology in operation showed sinusoid tumor. The postoperative pathological immunohistochemical results indicated CD56（+），CgA（+），Ki67（+about 2%），PanCK（－），PR（－），S-100（scattered+），Syn（+），TTF1（－），PD-L1（22C3）（about 40%tumor cell positive）. Combined with the clinical and immunohistochemical results， pulmonary metastatic PGL was considered.The genetic test was done by using the surgical specimens， no gene mutation was found，there are only a few enzymatic polymorphism related gene mutation including CDA，DPYD，ERCC2，GSTM1，MTHFR and XRCC1；MSI-H was not found； the tumor mutational Burden （TMB） was 0 mutation/1 million bases. After surgery， the patient decided to get further treatment in other hospital.

PGL is a rare malignant neuroendocrine tumor， the clinical manifestations are not typical，the patients often come to hospital because of metastatic lesions， and pulmonary metastasis is relatively rare in the PGL patients which leads to misdiagnosis. Pathological examination is important to make a definite diagnosis.