Pure gonadal dysgenesis with microscopic ovarian streak and gonadoblastoma.

A phenotypic female with an XY karyotype and no grossly demonstrable streak gonads is described. She had a microscopic focus of gonadoblastoma developing in a 3 X 2 mm area in the region of the ovarian vessels. The case stresses the importance of a careful search of the pelvic cavity in phenotypic females with a 46, XY karyotype.