Objective To explore the clinical characteristics, diagnose and treatment of bronchiolitis obliterans (BO) associated with lymphoma and paraneoplastic pemphigus (PNP). Methods One patient with BO associated with lymphoma and paraneoplastic pemphigus was presented and literatures which searched through Wanfang Med online and PubMed on this subject were reviewed. Results A 44-year-old woman was admitted to this hospital because of oral ulcer for two years, and persistent dyspnea for fourteen months. She had been diagnosed with PNP one year earlier. Pulmonary function displayed severe obstructive ventilator dysfunction, negative response to the bronchodilator. Chest CT showed bronchiolectasis. The ratio of neutrophils increased in bronchoalveolar lavage fluid. The pathology of ultrasound-guided neck and inguinal lymph node biopsy showed non-Hodgkin’s lymphoma. Based on the clinical presentation, the image and pathology of this patient, the diagnosis of having PNP with BO secondary to the lymphoma was made. After 10 cycles of CHOP chemotherapy, lymphoma and PNP went into remission, but the patient died from respiratory failure. The literatures were reviewed and 15 patients were found, out of them 60% (9/15) were female, 47% (7/15) follicular lymphoma and 47% (7/15) diagnosis of clinical BO which did not be supported by pathology. Lymphoma-PNP-BO was a clinical syndrome and 67% of the initial presentations were PNP. Patients died within 7 to 60 months. Conclusions BO associated with lymphoma and PNP is rare and the most common initial presentation is PNP. BO is the major cause of death. Treatments included chemotherapy, corticosteroid and immunosuppressor, lymphoma and PNP can be alleviated, but BO is aggravating.