Background: There are 11 reported cases of pregnancy in true hermaphrodites, but none with advanced genetic testing. All known fetuses have been male.
Case: A true hermaphrodite with a spontaneous pregnancy prenatally known to have a remaining portion of a right ovotestis, delivered a male neonate. The mother has a 46, XX karyotype with polymerase chain reaction demonstrating low levels of the Yq12 sequence. Postpartum androgen levels were normal.
Conclusion: Partial removal of testicular tissue may enhance fertility in hermaphrodites, and there may be a genetic basis for the progeny to be male.