Objective:The objective of this study is to investigate the clinical manifestations, computed tomography (CT) findings, and prognosis of primary pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) with the improved health awareness nowadays.Methods: Clinical data and CT images of patients with pathologically confirmed MALToma from October 2012 to October 2021 were retrospectively analyzed.Results: Eighteen MALToma patients were included in the study (8 men and 10 women; mean age, 56.17 years; range, 33-73 years). Six men had a long-term history of smoking (>20 years). One patient had an autoimmune disease (Sjögren syndrome). Eight patients (44.44%) were asymptomatic, and 10 (55.56%) had mild chest or systemic symptoms. Most lesions (88.89%) were subpleural or located along the bronchovascular tree. According to the CT characteristics, the lesions were divided into 4 groups: nodular type (n = 8; 44.44%, including 5 solid nodules and 3 ground-glass nodules), mass type (n = 4, 22.22%), patch or consolidate type (n = 5, 27.78%), and mixed type (n = 1, 5.56%). Air bronchogram (11/18, 61.11%) and angiogram sign (8/16, 50%) were the most frequent CT findings and may help differential diagnosis. The patients were misdiagnosed with lung cancer (n = 11, 61.11%), inflammatory or chronic inflammatory disease (n = 4, 22.22%), bronchiectasis accompanied by infection (n = 1, 5.56%), diffuse interstitial lung disease (n = 1, 5.56%), and granuloma or tuberculosis (n = 1, 5.56%). The prognosis of all patients was favorable; 1 patient was lost to follow-up.Conclusions: Nowadays, patients with MALToma are usually asymptomatic or paucisymptomatic. Nodular lesions, including ground-glass nodules, are the most common presentation. Mucosa-associated lymphoid tissue lymphoma is most likely misdiagnosed as lung cancer. Lesions located in the subpleural areas or along the bronchovascular tree and presenting with an air bronchogram or angiogram sign could indicate a MALToma diagnosis.