The aim of this paper is to determine, compare and summarize presentation characteristics of patients with lymphoma of the oral cavity and maxillofacial region using the results of different primary studies. Articles from the PubMed database published between 1990 and 2010 were reviewed. The key words "Lymphoma" and "Oral" were used to search for titles, identifying 215 articles, of which 178 were excluded and 37 were used for this review. After evaluation, the researchers agreed to include 15 of the papers in the study. This paper describes the author year published, number of cases, sex, age, location, signs and symptoms, classification, diagnosis, staging and treatment reported in each of the 15 studies. A total 714 patients are described. In 11 out of 13 studies, there were more cases in males than females. Ages ranged from 3 to 96 years. Presentation was most frequently the gingiva and the main sign was swelling. Staging was described for only 177 cases and treatment was described for only 110 cases. It is concluded that the presentation of most of the lymphomas of the oral cavity and maxillofacial region is extranodal, non-Hodgkin's. The most common locations are gingiva in the oral activity and Waldeyers's ring in the maxillofacial region. Although these lymphomas are rare pathological entities, it is important to describe the complete manifestation of their natural history in order to provide knowledge of their development.