RESUMO
Intersex is a general term used for a variety of conditions in which a person is born with a reproductive or sexual anatomy that doesn't fit the typical definitions of male or female. Androgen Insensitivity Syndrome (AIS), formerly known as testicular ferminization, is one that falls into this category. AIS is an X-linked recessive condition resulting in a failure of normal masculinization of the external genitalia in chromosomally male individuals. This paper presents the first recorded case of Androgen Insensitivity Syndrome in our institution in a phenotypically female patient with primary amenorrhea, physical examination findings of inguinal mass, full breast development, blind ending vagina, scanty pubic hair, absent uterus, absent ovaries, absent axillary hair and an XY karyotype. This paper also aims to discuss steps in the diagnosis and management of the condition, its legal impacts and the importance of psychological counseling for patients.