A 26-year female was admitted to hospital with fever and cough. Blood routine test showed leukopenia and thrombocytopenia. CT scan indicated pneumonia-like disease. Antibiotics therapy was ineffective, and primary pulmonary extranodal NK/T-cell lymphoma (ENKTL), nasal type was confirmed by percutaneous lung biopsy. Twenty cases of ENKTL were collected from 14 reports through literature retrieval. Patients aged 19-80 years with a male to female ratio of 3∶1. Main clinical symptoms included fever (85%), cough (65%), and shortness of breath (55%). CT findings of primary pulmonary ENKTL varied widely and might occur in all lobes of both lungs; the lesions were nodular or mass-like (60%) and pneumonia-like (20%). Few cases showed pleural effusion (25%) and/or mediastinal lymphadenopathy (25%). ENKTL presented NK/T cell phenotype, cytotoxic granule protein, and evidence of EB virus infection. The pulmonary ENKTL progressed rapidly, the hemophagocytic syndrome presenting with high fevers, hepatosplenomegaly or cytopenias usually indicated a late stage of the disease. The survival time ranged from 8 days to 12 months. The primary pulmonary ENKTL is a rare disease. The clinical course tends to be rapidly progressive, with life-threatening complications occurring less than a year after the disorder becomes apparent. When a non-responding pneumonia is associated with hemophagocytic syndrome (fever, leukopenia, splenomegaly), lung biopsy should be considered for the diagnosis of ENKTL.