A true hermaphrodite with some unusual features.

A fifteen-month-old boy with ambiguous external genitalia was found to have a 46XX karyotype, an ovotestis in the right labioscrotal fold, and an absent left gonad. He also had a rudimentary uterus and fallopian tubes and a blind-ending vagina lined with squamous epithelium. These features are compared with those more commonly found in true hermaphroditism.