Objective To explore the clinical characteristics, diagnosis and treatment plan of pulmonary lymphomatoid granulomatosis in order to deepen the understanding of this disease. MethodsA case of pulmonary lymphomatoid granulomatosis complicated with tuberculosis and human immunodeficiency virus (HIV) infection was reported. Literature reviews were searched in PubMed database with "pulmonary, lung, lymphomatoid granulomatosis" as the key words, and in China Knowledge Network and Wanfang database with "lung, lymphomatoid granulomatosis" as the key words. The search time was from January 1, 2017 to December 31, 2021. ResultsThe patient was diagnosed as pulmonary tuberculosis at the beginning of the disease, and the lesion was obviously absorbed and improved after regular anti-tuberculosis treatment. Six months after anti-tuberculosis treatment, chest CT examination showed multiple new circular nodules in both lungs. Intensive anti-tuberculosis treatment did not improve, further lung biopsy, pathology revealed lymphomatoid granulomatosis, grade 2; During the period, HIV infection was proven, and the patient underwent anti-viral infection and re-examination of chest CT lung lesions significantly improved absorption. Literature reviews found 47 same patients, therefore totally 48 patients were analyzed, in which this former case was included. Among the 48 patients, 26 were male (54.2%) and 22 were female (45.8%), with a median age of 60 years old (4 to 87 years old). The most common symptoms were cough, fever and shortness of breath, some of them may be accompanied with fatigue, weight loss, night sweats and loss of appetite. 20.9% of the patients had rashes, mainly manifested as erythema or papules. 39.6% of the patients were accompanied by immune system related diseases or immunosuppressants; The most common manifestations of chest CT were multiple nodules or masses involving both lungs. The main way of diagnosis was surgical lung biopsy, or CT-guided lung puncture biopsy. The positive rate of bronchoscopy biopsy was low. The pathological grade was mainly grade 3 (56.3%). The treatment plan was mainly R-CHOP, with an effective rate of 71.4%. For patients considered drug-induced disease, it was necessary to stop using induced drugs first, and then combined chemotherapy if there was no improvement. For HIV-infected patients, highly active antiretroviral therapy should be given first, if there was no improvement, then took combined chemotherapy; Of the 48 patients, 41 patients had clear follow-up results with a median follow-up time of 12 months, of which 14 patients were dead (34.1%), and the others got better in different degrees. Conclusions Pulmonary lymphomatoid granulomatosis is a rare disease. Clinicians should improve their understanding of it in order to identify the disease early, and choose the appropriate treatment scheme to improve its prognosis.