H-Y positive 46 XX true hermaphroditism with intrascrotal uterus

A rare form of true hermaphroditism with hypergonadotrophic hypogonadism, gynaecomastia, presence of an intrascrotal uterus, and 46 XX karyotype, is reported. It is the third published case in the literature since 1965. The presence of H-Y antigen and of testicular tissue in the absence of a Y chromosome is discussed.