Extranodal marginal-zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) arising in the thymus is rare, with the largest series in the literature including only three cases. In the present study, we investigated 15 cases of thymic MALT lymphoma to systematically characterize its clinical, histopathological, and molecular features. There was a marked female predilection (male:female ‫؍‬ 1:4), with a mean age of 55 years at diagnosis. There was a strong association with autoimmune disease, especially Sjögren's syndrome.