ABSTRACT

Complete Androgen İnsensitivity Syndrome (Testicular feminization) is the most common cause of male pseudohermaphroditism. The underlying pathology is an end organ resistance to androgen stimulation. Androgen Insensitivity Syndrome could be considered as a disease that causes resistance to androgens actions, influencing differentiation of the body structures and systems in which this hormone exerts its effects. Transmission of this disorder is by means of an X-linked recessive gene that is responsible for the androgen intracellular receptor. The disorder is often found in several members of the same family. This article presents the case of three sisters diagnosed with Complet Androgen Insensitivity Syndrom which was found out during the family history investigation of a female patient having a mass in her inguinal region and a diagnosis of Complet Androgen Insensitivity Syndrom.