IgG4-related sclerosing disease of the larynx (IgG4-SD) is a recently described immunodependent systemic pathology characterized by diffusive or focal inflammatory infiltration of the affected organs and tissues by plasma cells expressing IgG4; it is accompanied by the subsequent development of obliterative phlebitis and fibrosclerosis associated with the increase of the serum IgG4 level. According to the recently published materials, the disease can also develop in the respiratory system. The present article describes the first documented case of IgG4-related sclerosing disease with the isolated lesion of the larynx. The diagnosis was established based on the results of the comprehensive examination that made it possible to exclude systemic lesions, to determine the IgG4 level in the serum, and to carry out the immunohistochemical study of the histological preparations stained for the detection of IgG4-releasing plasmocytes. After verification of the diagnosis, the patient underwent a course of systemic hormonal therapy that resulted in the stable clinical and roentgenological remission of the disease that persists up to the present time.