Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of pulmonary origin is a relatively rare disease. In particular, reports of MALT lymphoma occurring and localized in the trachea or bronchus have been limited. Pulmonary MALT lymphoma has been reported to demonstrate spontaneous regression, whereas there is only one reported case of spontaneous regression of primary endobronchial MALT lymphoma. We herein report the case of a 70-year-old man with primary endobronchial MALT lymphoma who showed spontaneous regression with an interest of endobronchial findings.