Follicular lymphoma represents the second most common subtype of non-Hodgkin lymphomas. It is a neoplasm derived from germinal center B-cells that most often affects the lymph nodes. The translocation involving the BCL2 gene is considered the genetic hallmark of the neoplasm, present in approximately 90% of the cases. Follicular lymphoma affecting the oral cavity demands systemic evaluation of the patient to investigate a possible disseminated disease. The palate and the cheek mucosa are the most affected locations. Males and females are equally affected, and adults ranging from the third to the seventh decades of life are the most affected. The tumors usually present as expansive, non-ulcerated, painless swellings with a long evolution. In addition to its classic presentation, several other variants of follicular lymphoma are recognized and should be ruled out before diagnosis can be confirmed. Follicular lymphoma demonstrates a follicular growth pattern with predominance of small cleaved cells and a variable number of large cells. The tumor expresses germinal center markers like CD10 and BCL6, and strongly expresses BCL2 in the neoplastic follicles. The use of rituximab with the CHOP scheme represents the main therapeutic approach, which led to a significant improvement in the prognosis and survival of affected patients.