Ovotestis refers to the histology of a gonad that contains both ovarian follicles and testicular tubular elements. Such gonads are found exclusively in people with ovotesticular disorder of sexual development (OT-DSD), formerly known as true hermaphroditism. Those diagnosed with this rare condition represent a small fraction of patients within the diagnostic category of the disorders of sexual development (DSD), formerly known as intersex. Within the spectrum of DSD, there are varying degrees of discordant genitalia to sex chromosomes. A diagnosis of OT-DSD is based solely on the presence of ovarian and testicular tissue in the gonad and not on the characteristics of the internal and external genitalia, even if ambiguous.