ABSTRACT
Androgen insensitivity syndrome is a genetic syndrome characterized by complete or partial resistance of end organs to the peripheral effect of androgen. The phenotype of this condition is female, despite the normal male karyotype 46,XY. This is the third most common cause of primary amenorrhea after gonadal dysgenesis and mullerian agenesis. There is a congenital insensitivity to androgens, transmitted by means of a maternal X-linked recessive gene responsible for the androgen intracellular receptor. Prophylactic gonadectomy in usually performed due to increased risk for development of malignancy and possible virilization. Pelviscopy provides a minimally invasive technique for the accurate diagnosis and also provides the opportunity for therapeutic management of these patients. Recently we experienced two cases of this syndrome and removed the gonad by pelviscopic surgery, so we presented them with brief review of literature.