Abstract: Objective To explore the clinical manifestations and pathological features of ocular adnexal lymphoma. Methods The clinical and pathological information of 27 patients diagnosed as ocular adnexal lymphoma in 81 Hospital of PLA by pathological examination from January 1990 to December 2013 were collected， and the pathological features of the disease were analyzed retrospectively. Results The lesions were limited to the orbital area in 25 cases and the rest 2 cases were secondary to the lymphoma of other organs. The pathological diagnosis in all the cases was non-Hodgkin's lymphoma， of which T cell in 2 cases and B cell lymphoma in 25 cases including mucosaassociated lymphoid tissue（MALT） lymphoma 13 cases， lymphoplasmacytic lymphoma（LPL） 4 cases， mantle cell lymphoma（MCL） 3 cases，small lymphocytic lymphoma（SLL）2 cases，extramedullary plasmacytoma（EMP） 3 cases. There were 13 cases originally diagnozed as small B cell lymphoma. They were clearly diagnozed as 8 MALT lymphoma, 3 MCL and 2 SLL by immunohistochemical staining. Conclusion Clear classification can provide more useful suggestions about the choice of treatment and prognosis for the patients.