Marginal zone lymphoma is a group of low-grade B-cell neoplasms that account for approximately 7%–8% of all non-Hodgkin lymphomas. Five different marginal zone lymphomas are recognized, including splenic marginal zone lymphoma, nodal marginal zone lymphoma and extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma), pediatric nodal marginal zone lymphoma and primary cutaneous marginal zone lymphoma. MALT lymphoma represents the most common subtype, more often observed among adults in their fifth to sixth decades of life. It may affect many different extranodal locations, especially the stomach, ocular adnexa and lung. In the head and neck, MALT lymphomas are more common in the major salivary glands, manifesting as painless swellings. In the oral cavity, adult females and the palate are the most affected, usually as a solitary, asymptomatic and non-ulcerated nodule. MALT lymphoma demonstrates a diffuse proliferation of small to medium-sized neoplastic cells, aggregates of monocytoid cells and the presence of lymphoepithelial lesions. There is no specific marker for this lymphoma, and diagnosis demands the exclusion of other small B-cell lymphomas. Treatment varies from chemotherapy, surgery, immunotherapy, radiotherapy, or watch and wait approaches, and prognosis is usually good, with a median survival exceeding 10 years.