Follicular lymphomas represent the second most frequent lymphoma subtype. Translocation t(14;18)(q32;q21) is a characteristic biologic hallmark. It is not sufficient to drive follicular lymphomas development and subsequent molecular defect appears which lead to follicular lymphomas development and progression. The microenvironment plays an important role. The disease is usually diagnosed in an advanced clinical stage. The course is mostly indolent, but there is a subgroup characterized by rapid progression. The outcome has been improved with median of progression free survival between 6-7 years and overall survival between 10 and 15 years. The outcome improvement was caused by introduction of immunotherapy - rituximab, both in induction as well as in maintenance therapy. Despite this improvement, subsequent relapses occur, they can be managed by a variety of approaches based on many factors. The most adverse event is histological transformation. The present review briefly summarizes understanding of biology, clinical course and management.