Follicular lymphoma (FL) is the second most common lymphoid tumor. It is composed of elements resembling those of normal germinal centers. In particular, it is constituted by small centrocytes and large centroblasts, typically CD10+, CD19+, CD20+, CD79a+ and BCL6+, with follicular growth pattern. The molecular hallmark of FL is the t(14;18)(q32;q21) translocation, which leads to inappropriate BCL2 expression. This feature, other than representing a pathogenetic primary event, constitutes a suitable diagnostic marker, as well as a target for minimal residual disease monitoring and, hopefully, future therapies. Clinically, FL presents with indolent behavior, characterized by prompt response to initial therapy but almost invariably subsequent relapses. Novel approaches, including stem cell transplantation, monoclonal antibodies and innovative agents, should be then considered for improving long-term results.