We report a case of thymic mucosa-associated lymphoid tissue (MALT) lymphoma (TML) that presented as an asymptomatic mediastinal mass in a 40-year-old woman with a past history of Sjögren syndrome. This case had the characteristic clinical and pathological features of TML, as found in most of the 24 previously reported cases, i.e., autoimmune context, especially Sjögren syndrome, IgA secretion, large epithelial cysts, lymphoepithelial lesions involving residual Hassal's corpuscles, epithelial cysts, and a marked plasmacytic differentiation with IgA expression. Reverse-transcription polymerase chain reaction for t(11;18)(q21;q21) was negative, in agreement with recently published data. In this case, investigation for t(14;18)(q32;q21) using fluorescent in situ hybridization was also performed and supplied negative results. Neoplastic cells were negative for MAL, a marker of primary mediastinal large B cell lymphoma (PMBL). Altogether, these findings further support that among MALT lymphomas, TML have peculiar clinical and morphological characteristics and appear not to involve MALT1 rearrangement. They also suggest the absence of a relationship between TML and PMBL.