Objective To investigate the clinical data of a patient with IgG4-related disease involving the trachea and paratracheal soft tissue and review the literature so as to improve the understanding level of the disorder.Methods To analyze the clinical manifestation,laboratory examination,imaging,histopathology,treatment and prognosis of a patient with IgG4-related disease trachea and paratracheal soft tissue involved,who was admitted to the Department of Respiratory and Critical Care Medicine at Beijing Chaoyang Hospital.The relevant literatures were reviewed.Results A 18-year-old female was admitted with chief complaint of cough,dyspnea,and neck mass.Neck CT suggested that tracheal stenosis was caused by surrounded soft tissue.Paratracheal mass biopsy showed dense collagen fibers with infiltration of many lymphocytes and plasma cells.Immunohistochemical stain found that IgG4-positive plasma cells were ＞ 50/high power field (HPF) and a ratio of IgG4/IgG positive cells was over 40％.The level of serum IgG4 was significantly increased (2 930 mg/L).She was diagnosed as IgG4-related disease.The patient was treated with 80 mg intravenous methylprednisolone per day for three days,then prednisone 40 mg daily oral.Her dyspnea was significantly relieved.One month later,CT scan showed that the cervical tracheal stenosis was significantly improved.We identified 20 cases of IgG4-related disease involving the trachea and paratracheal soft tissue from databases,in which only 1 case was similar as this patient.The other 19 cases were of extratracheal involvement.Elevated serum IgG4 was detected in 11/12 patients.Most patients were treated with glucocorticoid,some combined with immunosuppressive agents and rituximab.The clinical outcome was good.Conclusion IgG4-related disease involving the trachea and paratracheal soft tissue is a rare condition.Serum IgG4 level and histopathology should be considered for diagnosis.Glucocorticoid is effective.