Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of the thymus is rare and little is known about its karyotype abnormality. MALT lymphoma in general shows a good prognosis, but some reports suggest that the presence of trisomy 18 predicts recurrence. Here, we report a patient with MALT lymphoma of the thymus and the left parotid gland accompanied by Sjogren's syndrome. The karyotype analysis revealed that this is the first case of thymic MALT lymphoma with trisomy 18, which we believe is worth reporting. We also review cases with thymic MALT lymphoma previously reported in the literature.