Extranodal non-Hodgkin lymphoma (NHL) is infrequent in the oral cavity and constitutes 3.5% of oral cancers, and less than 2.2% of maxillofacial lymphomas. Diffuse large B cell lymphoma (DLBCL) accounts for 40% of NHL and has a 5-year survival rate of less than 30%. Early detection of extranodal NHL by dental personnel is extremely important as a delay in diagnosis can result in the cancer being diagnosed at an advanced stage and a poor prognosis. A 60-year-old male presented with an uncharacteristic asymptomatic rapidly enlarging swelling of the anterior maxilla, which on histopathological and immunohistochemical analysis was diagnosed as DLBCL. Imaging studies showed bone invasion and lymph node metastasis with poor prognosis. The patient received radiotherapy and chemotherapy but died within 3 months of diagnosis. A literature search revealed one another case with anterior maxilla occurrence, as the few oral DLBCL so far reported have appeared on the posterior palate or other intraoral sites.