English

Abstract

Testicular feminization syndrome or androgen insensitivity syndrome is a rare disorder. The individual with complete form of this syndrome (CIAS) have female external genitalia while those with partial form (PIAS) have variable ambiguity of genitalia and often need extensive reconstructive surgery. The etiology of this syndrome is congenital insensitivity to androgens transmitted by means of a maternal X-linked recessive gene responsible for androgen intracellular receptors. We have found an un-usual presentation of female phenotype with androgen insensitivity syndrome, which is discussed.