Abstract: Objective To analyze the clinical characteristics and therapy of androgen insensitivity syndrome (AIS). Methods A retrospective analysis about clinical data,pathology and treatment was performed in 15 AIS patients admitted to the First Medical Center of Chinese PLA General Hospital from January 1993 to December 2019. Results Totally 10 patients with complete androgen insensitivity syndrome (CAIS) presented with female appearance.The main manifestations were primary amenorrhea in 7 cases,abdominal mass in 2 cases,and vulva infantilism in 1 case.Other 5 patients with incomplete androgen insensitivity syndrome (IAIS) presented with external genital abnormalities (hypospadias and clitoral penis in 4 cases) and short penis combined with breast development in 1 case.The average age at first consultation was (18.2±5.3) years and the puberty initiation was (14.9±2.1) years old.The serum testosterone,luteinizing hormone and follicle stimulating hormone were (30.6±16.3) nmol/L,(22.5±10.3) mIU/ml and (13.6±8.3) IU/L,respectively.Nine cases with CAIS accepted sex gland resection,in which 4 patients had estrogen replacement therapy to maintain secondary sexual characteristics;The gonad pathological examination showed dysplasia of seminiferous tubule and interstitial cell hyperplasia. Conclusion AIS is a rare sexual differentiation disorder,with complex clinical manifestations.Early identification and multidisciplinary collaboration is necessary for precision therapy and better prognosis.