It is an abnormality of male sexual differentiation with a prevalence of 1 in 20,000 to 1 in 64,000 male births. It is an abnormality of male sexual differentiation with a prevalence of 1/20,000 to 1/64,000 of newborn boys, in which androgen production and metabolism are normal, but the target organ does not respond to testosterone or dihydrotestosterone due to mutations in the androgen receptor gene (AR). Androgen activity in target cells is mediated through the classical and complex steroid receptor pathway, and any AR gene alteration that impairs androgen activity results in androgen insensitivity syndrome. As of 2008, three hundred AR mutations have been identified and 70% of AR mutations in AIS cases are familial X-linked recessive. androgen receptors are functionally or quantitatively abnormal in AIS patients and fail to mediate androgen stimulation properly, resulting in abnormal external genital masculinization. Depending on whether the androgen receptor is completely or incompletely abnormal, it can be subdivided into complete androgen insensitivity (CAIS) and incomplete AIS (partial vs. mild) and spinal muscular atrophy (Kennedy’s disease). Complete AIS presents with female external genitalia but no female internal genitalia ducts, elevated testosterone at puberty with abnormally elevated LH, but impaired spermatogenesis and organismal masculinization. Patients with incomplete AIS exhibit varying degrees of hermaphroditism, and Wilson et al. referred to incomplete AIS as incomplete male pseudohermaphroditism type I. The mild form can be phenotypically male but sterile. Complete androgen insensitivity syndrome is distinguished from partial androgen insensitivity by a degree of feminization or feminization of the male with cryptorchidism, hypospadias, micropenis, and scrotal splitting. Mayer-Rokitansky-Ku¨ster-Hauser (MRKH) syndrome is a common cause of primary amenorrhea and is due to a Mullerian developmental defect. It is characterized by normal breast development but normal testosterone, absence of vagina, and normal axillary and pubic hair. The key to diagnosis is karyotyping. swyer’s syndrome and XY complete gonadal dysgenesis both have no breast development, and imaging tests such as ultrasound and MRI can help differentiate. Patients with simple gonadal dysgenesis have female external genitalia, infantile uterus, fallopian tubes and striated gonads. Secondary sexual characteristics do not develop because the striped gonads do not produce normal levels of estrogen androgens. Also estrogen deficiency will result in mammary gland dysplasia, incomplete pelvic-hip extension and absence of menstruation, which can be differentiated from AIS. It should also be differentiated from partial gonadal dysgenesis, 17B-hydroxysteroid dehydrogenase deficiency, 5a reductase deficiency, and mixed gonadal dysgenesis with mosaic Turner’s syndrome (45xo, 46 Xy). A patient was treated for a complete androgen insensitivity syndrome with a concentrated number of cases in the family line. Clinical features: the patient has no uterus, fallopian tubes, or proximal vagina due to testicular production of Müllerian inhibitory factor (MIF). A short segment of vagina is present distally. The urogenital tract is devoid of wolffian and müllerian duct-derived structures and prostate glands. The testes are located in the abdominal cavity or groin, or descend into the labial folds. The testes are often seen in early childhood for “inguinal or incarcerated hernia”. The literature reports that 1.1% of girls with a clinical diagnosis of inguinal hernia may be completely androgen insensitive. The absence of uterine and ovarian development often leads to primary amenorrhea in adulthood. Testosterone is elevated during puberty, but masculinization is hampered by complete androgen insensitivity; at the same time, LH and estradiol are commonly elevated, giving the patient a feminine phenotype and a female external genital appearance. There is mammary gland development but little nipple pigmentation and absent or sparse axillary and pubic hair. Because of its female phenotype, complete androgen insensitivity syndrome is preferable to female rearing. Patients without testicular dysplasia are at low risk of developing testicular tumors before puberty, and the testes may be withheld from removal so that they have sufficient estrogen to promote feminization. However, the risk of developing gonadal tumors after puberty is about 0.8% to 5%, and close follow-up is needed. After puberty, it is advisable to remove the testes and administer low-dose estrogen replacement therapy to maintain feminization and avoid osteoporosis. Corresponding surgical treatment is feasible for those with clinical manifestations of inguinal hernia. Advances in laparoscopic instruments and techniques have minimized the invasiveness of hernia surgery, and gonadal exploration can be performed simultaneously with inguinal hernia surgery. Patients with short vaginal segments can be vaginally dilated or vaginoplasty can be performed in adulthood. The AR gene is localized on the long arm of the X chromosome (Xq11-12). The types of mutations include gene deletions, splice site mutations, early termination codons, and missense mutations. The CAIS gene assay is performed to confirm the clinical diagnosis on the one hand and to distinguish carriers on the other. In the present case, which is X-linked recessive, 50% of 46,XY offspring are AIS and 50% of 46,XX offspring are carriers. Carriers of childbearing age should pay attention to prenatal diagnosis or prenatal genetic diagnosis to identify fetal chromosomal karyotype. If prenatal ultrasound examination of the fetus is female possible, but the amniotic fluid cell karyotype is 46,XY, suggesting a patient, parents need to choose carefully for eugenics. The female phenotype of AIS patients with karyotype 46, XY gives them genetic sex, gonadal sex and body sex inconsistency and face identity barriers. Moral support from family members and professionals is also very important. Psychological support is an important part of treatment and should be communicated to patients at the right time and in the right way to help them adjust their mindset, understand their situation correctly, and reduce the risk of psychological problems; it also helps them grow up to be healthy adults.