Salivary gland mucosa -associated lymphoid tissue lymphoma is associated with autoimmune diseases and chronic infections, such as Sjögren syndrome, chronic autoimmune disease, hepatitis C virus, Epstein -Barr virus, herpesvirus, human T -cell lymphoma virus, human immunodeficiency virus. Chromosome translocation has seldom been observed in this disease, but it relates to definite chemokines, showing a conserved pattern of chromosomal gains, which appear to target genes positively modulating cell survival and proliferation, and has a background of benign lymphoepithelial lesion. It shows a chronic, painless clinical course. A long-term follow-up and repeating biopsy are needed to diagnosis. Radiotherapy and chemo are both responsive, and disseminated disease at multiple sites need therapeutic alliance. It has a good prognosis, although with a higher relapse rate than oher type of mucosa-associated lymphoid tissue lymphoma, and salivary gland swelling, myoepithelial sialadenitis, second-cancer and metastasis may also take place, even involves bone marrow. A long-term and attentive follow-up is important to the prognosis.