Follicular lymphoma (FL) is a heterogeneous entity with disparate outcomes based on clinical and pathologic characteristics. An increasingly detailed understanding of high-grade FL (grade 3) has led to the identification of separate categories of FL3A and FL3B. Recently, genomic studies have made much progress in delineating the genetic differences between FL3A and FL3B. Although a general consensus exists that FL3B follows an aggressive course matching that of diffuse large B-cell lymphoma, there is less certainty regarding the course of FL3A. Uncertainty also exists regarding the management of high-grade FL. Given that a majority of the prospective landmark trials in FL have excluded patients with high-grade FL, most of the available evidence is retrospective. This review summarizes the recent advances in the management of high-grade FL.