Abstract: To review and study the prognostic factors of a panel of extragastrointestinal primary mucosa-associated lymphoid tissue ( MALT ) lymphoma. Methods: Twenty-nine diagnosed cases of extragastrointestinal primary MALT lymphoma with complete clinical information from Cancer Hospital CAMS over a 10-year period were enrolled, and the correlations between their ages, primary site, histological feature, stage, therapeutic measures, and survival rate were retrospectively compared. Results: 1) The ages of the 29 patients ranged from 29-80, with a median of 55; the ratio of male to female was 1:3. The primary symptom was a local mass. Up to 9 ( 31% ) patients were infected with HBV ( P < 0.05 ). 2) Histologically, 27 cases had typical histologic and immunophenotypic characteristics, 14 cases had plasmacyte differentiation with or without a high-grade component. The clonal arrangement of B lymphocyte receptor gene occurred in 3 cases. 3) The total 5-year survival rate of IE, IIE, and IVE based on the Ann Arbor staging system were 92%, 100% and 69%, respectively, and the survival rates of IE and IIE were clearly higher than that of IVE. The differences in primary sites, therapeutic measures, and morphology had no influence on survival rate ( P > 0.05 ). Conclusion: Extragastrointestinal primary MALT lymphoma is an indolent lymphoma with unclear pathogenesis. The prognosis of the lymphoma was closely related to clinical stage. HBV infection may involve the carcinogenesis of the liver. Different primary sites, therapeutic measures, and morphology had no significant effect on the prognosis.