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Androgen insensitivity (testicular feminization) syndrome is a rare inherited form of male pseudohermaphroditism that occurs in a phenotypically normal woman with male karyotype (XY). We present a case of a 20 years old patient who had history of primary amenorrhea. The clinical examination shows a female phenotype: the breasts are normally developed, absence of hair in the groins and axillary areas, short blind vagina, absent cervix and uterus. A firm mass measuring 5x3 cms was palpable in the left inguinal region. MRI pelvis revealed a cystic lesion measuring 6.8x5.5 cms on the right side. Histopathological examination revealed that both the masses were composed of testicular tissue with absence of ovarian component. The testes were removed to avoid the risk of malignancy. The patient was counseled and discharged with lifelong hormone replacement therapy.

Complete Androgen Insensitivity Syndrome (Testicular Feminization Syndrome): An Interesting Case of Primary Amenorrhoea with 46XY Karyotype