Abstract: Folliculotropic mycosis fungoides (FMF) is a rare variant of mycosis fungoides, and is characterized by histological characters of perifollicular infiltration and folliculotropism of atypical lymphocytes. It have previously been reported that FMF had a worse prognosis than classic MF.  However, recent studies have shown that FMF can be divided into two different subtypes. Patients with patches, follicular papules and acneiform lesions are defined as early-stage FMF with favourable prognosis. The patients with infiltrated plaques, nodules, tumors, and/or extracutaneous involvement are defined as late-stage FMF with poor prognosis. Management of FMF patients should follow a stage-adapted therapeutic approach like classic MF. In this paper, the classification, prognostic factors and therapeutic options are reviewed.