Introduction: Androgen Insensitivity Syndrome (AIS) is a rare X-linked disorder characterised by an impaired virilisation in an individual with 46, XY karyotype. It is caused by several mutations in the Androgen receptor gene. There are 3 main phenotypes seen in AIS: Complete, partial and mild AIS. AIS can be difficult due to late diagnosis in puberty as well as due to the lack of clear guidelines present. This systematic review looks at the diagnosis and management of AIS in children. 

Methods: A systematic search was conducted looking into the process of diagnosis of AIS and the surgical and non-surgical interventions available to treat AIS. The studies were collated mentioning the year, type of study and the number of patients involved. The review also includes studies that looked at adults >18yrs due to limited literature on children. 

Results: A total of 28 articles were identified that fit the inclusion criteria. 

Conclusion: The main aim of the management of AIS should address the full disclosure of diagnosis, functional, sexual and psychological issues associated with it. The diagnosis of AIS may be made before or after puberty using karyotyping, ultrasounds and MRI scans. The main surgical intervention includes a gonadectomy followed by oestrogen replacement therapy especially for Complete AIS and PAIS after puberty. Mammoplasty for the reduction of gynecomastia can be offered to those with PAIS/ MAIS that identify as male. It is also important to involve the MDT and the family whilst making decisions about gender