Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare type of lung cancer with special characteristics of epidemiology, clinical diagnosis, treatment and prognosis. It has close relationship with Epstein-Barr virus (EBV) infection and has prominent regional feature. Most patients are young and non-smoking. There is no specificity of clinical manifestation. Most patients are asymptomatic at the time of diagnosis. As for treatment, the standard treatment for early stage disease is complete resection. Platinum-based doublet chemotherapy has been applied in locally advanced disease as the first line therapy. Due to its rarity, the treatment of advanced PPLELC is still lack of evidence of large sample randomized controlled trial. Whether target therapy or immunotherapy is effective is worth further study. This article reviews the research progress of PPLELC, to give a particular sight for clinicians and provides a better understanding of this rare tumor for researchers.