The pathogenesis of this condition, in view of a normal XY sex chromosome complement, must be complete testicular degeneration sometime between 6 and 12 weeks of pregnancy. If testicular loss is early, there is inadequate androgen stimulation (low wolffian development and female external genitalia), and mullerian ducts are preserved. The presence of a normal vagina, uterus, and tubes distinguishes this syndrome from testicular feminization variants. However, if testes loss occurs late, no gonads will be present, external genitalia will be ambiguous (but primarily female), and rudimentary ingredient of both mullerian and wolffian internal ducts will be present. Surgical removal of the streaks is required as a precaution against neoplasia.