All India Congress of Obstetrics & Gynaecology, 04–08 Jan 2009 · Birla Auditorium, Jaipur

Androgen insensitivity syndrome (AIS) is a disorder of sexual differentiation with 46XY karyotype. The gonad is at a risk (33% by 50 years) of developing malignant tumors. Hence gonadectomy is warranted. We present a case of 22 year, tall, beautiful, married female diagnosed as AIS, during investigation of primary amenorrhea. Ultrasound showed infra abdominally located gonads, with large non tender cyst of 9.2 x 5.6 x 5.4 cm size, with limited mobility, to right of midline. There was also a partial septum, with wall thickness of 1 - 2 mm and containing clear fluid.