Follicular lymphoma (FL) is an indolent non-Hodgkin's lymphoma that remains an incurable disease for most patients. It is responsive to a variety of different treatments, however it follows a pattern of relapsing and remitting disease. Traditional therapeutic options for patients with untreated FL include expectant observation for asymptomatic and low tumor burden and multiagent cytotoxic chemotherapy for symptomatic and/or high tumor burden. Biologics have become an integral part of therapy with agents that target B lymphocytes, including monoclonal anti-CD20 antibodies and radiolabeled anti-CD20 antibodies. Treatment response to cytotoxic and biologic therapy is high initially; however, with subsequent treatments, response rate and remission duration typically decline and cumulative toxicities increase. The identification of novel targeted agents, use of stem cell transplantation, and new treatment combinations provide the opportunity to enhance patient outcomes. In this review, we critically examine standard treatment strategies for patients with newly diagnosed and relapsed or refractory FL and discuss established and emerging novel therapeutic approaches.