<b><i>Introduction:</i></b> Keratosis follicularis spinulosa decalvans (KFSD) is a rare, X-linked, hereditary disorder of keratinization, characterized by skin involvement and progressive scarring alopecia of scalp, eyebrows, and eyelashes. The diagnosis is helped by the particular clinical features, but pathology is mandatory. <b><i>Case Presentation:</i></b> We described a case of a female patient referred to the outpatient’s hair consultation of our department, in which we performed trichoscopy as a very useful tool for the diagnosis, followed by pathology that confirmed KFSD. <b><i>Conclusion:</i></b> In our article, we underlined the importance of trichoscopy for the diagnosis of this hair disease, with also a review of the literature on diagnosis and treatment.