Objective To study the clinical characteristics, diagnosis and treatment of primary pulmonary lymphoma. Methods A retrospective review of primary pulmonary lymphoma cases at a single institution from 2006 to 2008 was performed, and relevant literature was reviewed. Results Primary pulmonary lymphoma is a rare disease. The diagnosis was difficult because of the lack of specific characteristics. The most common symptoms were cough and fever. X-ray feature included solitary or multiple nodules and consolidation. Definite diagnosis was made by pathologic and immunohistchemical examinations. The recommended first-line therapy is chemotherapy. Conclusion Appropriate invasive biopsy is necessary for early diagnosis of primary pulmonary lymphoma