Idiopathic tracheal stenosis is a type of benign stenosis that possesses specific characteristics but is of unknown origin. It is a rare disease characterized by extensive fibrosis of a portion of trachea, and predominantly found in women. The lesion presents as circumferential fibrotic stenosis that usually occurs at upper trachea and the subglottic larynx, but lower trachea may also be involved. Diagnosis is made from the clinical characteristics accompanied by compatible pathologic features and by exclusion of other etiologies. Conservative management such as laser resection, dilatation and stent insertion can be tried initially, but surgical resection is recommended for definitive treatment due to frequent restenosis and maintenance problems of conservative approach.