Androgen insensitivity syndrome (testicular feminization).

Androgen insensitivity (testicular feminization) syndrome is a rare inherited form of male pseudohermaphroditism that occurs in phenotypically normal women with adequate breast development, normal external genitalia, a vagina of variable depth, absent uterus, and sparse or absent pubic hair and axillary hair. These patients have male karyotype (XY) and negative sex chromatin. The gonad (undescended testis) may be intraabdominal, inguinal, or labial [1]. The incidence of testicular feminization syndrome is reported to range between one in 2,000 to one in 62,400 [2]. The present case is the first one from Jammu and Kashmir State of India where chromosome study has been carried out and 46, XY karyotype has been detected in the phenotypic female. Case History