Primary pulmonary MALT lymphoma is a rare disease, and no standard treatments have been defined yet. In this study, 38 consecutive patients from single center were reviewed. Among 25 patients with localized disease, radical surgery were performed in 12 patients, and the other 13 patients had chemotherapy combined with (7 patients) or without (6 patients) radiotherapy. No significant difference in overall survival (OS) was found between patients who received surgery or not; however, patients treated with chemotherapy had superior progression-free survival (PFS) than those treated with upfront surgery (P = 0.032). Among the 12 patients who received radical surgery, 7 were given adjuvant chemotherapy and 1 patient had consolidation radiotherapy. No significant differences in PFS and OS exist between those who received adjuvant treatment or not (P > 0.05). For patients who received chemotherapy, PFS and OS were significantly better for those treated with cyclophosphamide-based therapy than fludarabine-based therapy. At a median follow-up time of 61.1 months, 5- and 10-year PFS rate was 70.0 and 43.0 %, respectively, and 5- and 10-year OS rate was both 81.0 %. In conclusion, we confirmed the indolent behavior and favorable outcome of this disease. In order to preserve lung function and reduce the risks associated with surgery, radiotherapy or rituximab in combination with alkylating drug-based chemotherapy should be considered as first-line option for pulmonary MALT lymphoma.