Primary pulmonary lymphoepithelioma-like carcinoma(PPLELC) is a rare subtype of non-small cell lung cancer that is closely associated with Epstein-Barr virus infection. PPLELC has unique pathologic and molecular characteristics, including high lymphocytic infiltration, high expression of programmed cell death-ligand 1, and low mutation rates of common driver genes. This article systematically reviews the epidemiological characteristics, clinical manifestations, pathological and molecular features, diagnosis and treatment strategies, and prognostic factors of PPLELC, aiming to provide reference for the clinical diagnosis and treatment of this disease.