Mucosa associated lymphoid tissue (MALT) lymphoma of the lung is a rare disease with an indolent clinical behaviour. This single centre retrospective analysis evaluates the treatment strategies and clinical outcome for these patients. A total number of ten patients (7 male/3 female) were identified between January 1997 and October 2005 and their records analysed. At diagnosis the patients presented with unspecific symptoms (cough, shortness of breath and lower respiratory chest infection) which were further evaluated. Six patients had stage IAE disease, two patients stage IIAE and in two patients disease was stage IV. The initial treatment consisted of surgery alone (3 patients), chemotherapy +/- rituximab (5/1 patients), single agent rituximab (1 patient) and wait & watch strategy (1 patient). After a median follow-up time of 3.4 years the overall survival was 90% at 3 years. In conclusion, our data suggest that most of the patients with MALT of the lung had localized disease which generally responded well to systemic or local therapy and resulted in favourable long-term outcome underlining the indolent course of this disease.