Abstract: Objective To explore the diagnosis and treatment, the clinical characteristic, as well as the pathological features, of a rare case of progressive nodular histiocytosis involving the larynx. Methods Patient case data, pathological sections were extracted and observed by immunohistochemical staining to summarize the characteristics of this case and to consult the literature related to PNH. Results The pathology of the patient in this case suggested a diffuse infiltration of spindle cells, and immunohistochemistry was positive for CD68 and CD163. The patient presented with supraglottic stenosis, thickening of the tracheal wall, and multiple nodules in the trachea. The patient had undergone a tracheotomy due to dyspnea. This disease involves the skin, and it manifests in multiple nodules and facial nodules that give rise to a “lion face” appearance. After diagnosis, treatment with anlotinib can delay the development of this disease. Conclusion The disease is diagnosed based on pathological examination. It can involve the larynx and cause breathing difficulties. Surgical removal of the facial nodules is not recommended unless body function is seriously affected.

Key words: Histiocytosis, Non-neoplastic proliferative skin disease, Non-Langerhans cell histiocytosis, Laryngeal