AbstractFollicular mucinosis (FM) is an epithelial reaction pattern characterized by follicular mucin accumulation. It has been described in association with various inflammatory and neoplastic cutaneous disorders. FM is generally divided into a primary benign idiopathic form and a secondary form usually occurring in association with cutaneous lymphomas (especially mycosis fungoides), among other entities. Distinction between the two forms can be challenging as they share many overlapping features and the lack of a single diagnostic tool to differentiate between the two. Making the distinction may require evaluating and correlating the clinical, histologic, immunohistochemical, and molecular studies together. Long‐term clinical follow‐up also remains very important. In this review, we describe the different entities associated with FM, its pathogenesis, and possible therapeutic options.