Abstract: Salivary glands mucosa-associated lymphoid tissue lymphoma(SGML) is a distinct subtype of marginal zone B-cell type non-Hodgkin′s lymphoma (NHL), which is commonly seen in middle aged females. SGML is usually associated with autoimmune diseases such as Sj?gren's syndrome or with chronic infection such as hepatitis C virus(HCV)infection.Chromosomal abnormalities are frequently seen in SGML, which usually activate nuclear factor-κB molecular pathway to modulate cell survival and proliferation, resulting in lymphoma occurrence. SGML tends to arise from parotid gland, presenting frequently as a localized and indolent lesion, a long-term follow-up and biopsy are needed for accurate diagnosis. Surgery, radiotherapy and chemotherapy are usually effective disseminated diseases at multiple sites need combined treatment.SGML has a relatively better prognosis with a higher relapse rate than other types of NHLs,dissemination or higher degree of malignant transformation may occur.Thus,a long-term and close follow-up is essential for patients with SGML.