Folliculitis decalvans is a difficult to treat rare neutrophilic dermatosis that affects the scalp and leads to permanent scarring. The pathogenesis is currently not well understood; the influence of S.aureus antigens, a disorder of the skin microbiome, a defect in cell-mediated immunity, and an imbalance of pro-inflammatory cytokines are assumed. This disease is often associated with a marked decrease in quality of life, because in addition to significant external manifestations, the patient may experience pain, itching and burning in the areas of the rash. The main problem is that there is currently no effective therapy for this disease. Treatment options include systemic antibiotics, topical and intralesional corticosteroids, dapsone, isotretinoin, biologics, and photodynamic therapy. We present a brief overview of the main methods of therapy and a clinical case of a long course of severe folliculitis decalvans, refractory to the main methods of treatment.