Abstract： Objective:To describe the clinical features, treatment and prognosis of primary pulmonary lymphoepithelioma-like carcinoma(PPLELC).Methods:We conducted a retrospective analysis of 18 patients diagnosed with PPLELC from January 2014 to July 2021 in the First Affiliated Hospital of Nanjing Medical University. Clinical symptoms, lab data, imaging characteristics, treatment and prognosis were reviewed and analyzed.Results:This study consisted of 10 men and 8 women, with a mean age of (56.4±6.3) years, and two of them had a history of smoking. Most of them had no symptoms, while others showed some symptoms including cough (4/18), chest tightness (2/18) or chest pain (1/18). Serum levels of carcinoembryonic antigen (CEA) were normal (17/17), while serum levels of neuron-specific enolase (NSE) (7/16) and CYFRA21-1 (4/16) were increased, respectively. According to the eighth edition of international TNM staging standard for lung cancer, 12 of 18 patients were classified as stageⅠ. The lesions were solitary, mostly in the right lung (10/18), and peripheral type (13/16) were common, which could be accompanied by lobularization (9/16) or cavitation (2/16). The PET/CT data of 6 patients showed that the maximum standardized uptake value (SUVmax) was (8.15±3.08) and the metabolic tumor volume (MTV) was (6.85±4.95) cm 3. Ten patients (10/18) received gene tests. Among them, 6 cases were detected for ALK, and only one of them was positive for ALK mutation. The pathology of PPLELC was similar to that of undifferentiated nasopharyngeal carcinoma and it should be differentiated from ordinary lung squamous cell carcinoma according to EBER detection. Surgical resection or surgery combined with chemotherapy and immunotherapy were the main treatments in this study. Up to the follow-up time, the average survival time of the 18 patients was (26.0±12.7) months, and only 1 patient died(overall survival time of 24 months), and the median overall survival (OS) was not reached, with a 2-year OS rate of 87.5%. Conclusions:PPLELC usually occurred in the right lung and belonged to the peripheral type. The gene mutation was rare. PET/CT is helpful for early diagnosis and most patients can be diagnosed in the early stage. Surgery and surgery combined with multimodality therapies are the main treatments.