Objective To investigate the clinicopathologic features, diagnosis, treatment and prognosis of the primary lymphoepithelioma-like carcinoma (PLELC) of the lung. Methods Clinical and pathological data of one patient with PLELC of the lung were reviewed. PLELC of the lung was analyzed through review of literatures. Results A mass in the upper lobe of the left lung and atelectasis were detected by CT in a 36 year old nonsmoker male. He presented with cough, expectoration, fever and shortness of breath for one month. Neoplasm in left main bronchus was observed through bronchoscopy. Histological study showed cohesive clusters of comprising syncytial-appearing cells with vesicular nuclei and tumor infiltrating lymphocytes. Immunohistochemistry showed positive expression of Epstein-Barr virus (EBV) encoded small nuclear RNA. Nasal CT scan was negative for suspicious nasopharyngeal mucosal lesion or tumor. Then he was diagnosed as PLELC. The patient received docetaxel+nedaplatin regimens for 6 cycles with stable disease, but one month later after the end of chemotherapy, he was readmitted to hospital for dyspnea. CT scans showed progression of the lesion with massive pericardial effusion and pleural effusion. The patient could not tolerate chemotherapy, then received supportive treatment and died soon. Totally 18 Chinese articles and 25 foreign articles were screened out. Literature review suggested that patients with PLELC of the lung usually occurred in young nonsmokers and without gender difference. PLELC of the lung was closely associated with EBV infection. Its clinical manifestations were not specific, so that histopathological and immunohistochemical analyses were the main method of diagnosis. PLELC of the lung may respond to chemotherapy and radiotherapy. Surgery-based multimodality treatment was recommended. Patients with early or resectable disease had better prognosis, but patients with unresectable disease had poor prognosis. Conclusions PLELC of the lung may be closely associated with EBV infection. Histopathological and immunohistochemical analysis is the main method of diagnosis. Surgery-based multimodality treatment should be recommended because of its high response to chemotherapy and radiotherapy. Patients with early or resectable disease have better prognosis.