Cause not known. James Benson may have had this syndrome, although his male gender identity is atypical. These boys usually develop a male identity even if castrated and surgically reconstructed as females. Anatomically male but infertile. Affected individuals have one X and no Y chromosome.
Ovaries degenerate during fetal life, in other respects anatomically female.
Short stature and lack of secondary sexual characteristics without treatment. Old medical term for an intersexed person, given new life by some intersex activists.