Androgen insensitivity syndrome is one of the important causes of male infertility and male hermaphroditism and is an X-linked recessive disorder, but 1/3 of patients have no family history. Androgen insensitivity syndrome is a general term for a group of genetic developmental disorders associated with androgen receptor (AR) defects, which are caused by multiple mutations in the androgen receptor gene, the most common being testicular feminization syndrome due to AR defects. Clinical manifestations: The manifestations and severity depend on the level of AR expression and the degree of functional impairment of the AR, thus the clinical manifestations of the disease are highly heterogeneous, ranging from complete feminization of the external genitalia to a completely male appearance (only sterility or gynecomastia). It can be divided into four types: 1. Complete testicular feminization, although all patients with this disease have a karyotype of 46, XY, some patients have cool female external genitalia and are not diagnosed until a mass is found in the groin (testes) or until puberty when mammary glands develop without menstrual flow. Their testes can also be located in the labia majora or in the abdominal cavity, and a few patients can even lead a sexual life as a female without fertility; 2. Incomplete testicular feminization, which can have varying degrees of feminization, from resembling a female vulva to resembling a male vulva with only hypospadias. In between can be manifested as bifurcated scrotum, sexual hair, body hair, mammary gland development is also closer to male than in patients with complete feminization, are infertile; 3, male mammary gland development, their external genitalia are male, after puberty there is progressive mammary development, similar to female breast, but no pain or overflow, with axillary hair is absent, the amount of semen is small, but the sperm viability is normal, the prostate is smaller than normal, the most mild type of the disease. 4, male infertility, the patient’s appearance is completely normal male, but the number of sperm is severely reduced resulting in infertility. Treatment: Includes correction of external genital deformities and hormone replacement to promote and maintain secondary sexual characteristics. Treatment options depend on the age of the patient, the severity of the external genital malformation, and the wishes of the patient and family. Depending on the degree of external genital malformation, surgical correction is performed to preserve the male or female genitalia, respectively, followed by estrogen and progestin or androgen replacement therapy, but neither can restore the reproductive function. Prevention: 1. Perform prenatal diagnosis; 2. Gender oriented surgical treatment should be performed before the patient establishes the concept of gender and give focused gender education after the concept of gender is established; provide education about the disease to patients who are determined to be raised as females to reduce the ideological pressure on the lack of fertility.