The rarest cases of human hermaphroditism are the instances of true or gonadal hermaphrodite. In this condition each gonad is an ovotestis - part ovary and part testis - or one is an ovary and the other a testis. Sawyer, writing in 1954, remarks that only forty cases had been recorded in all the medical literature. The condition can be diagnosed only by microscopial examination of parts of the gonads.

Among the outward signs in a "male" are hypospadias, undescended testicles and abnormal breast development, in the "female" with this condition there is abnormal development of the clitoris. Normal spermato-genesis may occur close to an ovary in which ovulation takes place.