46, XY gonadal dysgenesis with secondary amenorrhea, virilization, and bilateral gonadoblastomas

A phenotypic girl with secondary amenorrhea, enlargement of the clitoris, XY gonadal dysgenesis, and bilateral gonadoblastomas is described. The presence of secondary amenorrhea does not obviate the existence of a Y chromosome. The presence of the Y chromosome should be a warning that a gonadal tumor may be present and, therefore, gonadectomy must be done as soon as possible and preferably before puberty.