Gender Dysphoria in a Patient With Ovotesticular Disorder of Sex Development.

ABSTRACT

Ovotesticular disorder of sex development (OT-DSD) is a rare condition characterized by the presence of both ovarian and testicular tissue in the gonads. Management and sex designation of these patients depend on several factors, and an underlying potential for gender dysphoria should be acknowledged. We present a case of a patient diagnosed with 46,XX OT-DSD at 12 months old who was attributed a female sex designation but started manifesting gender dysphoria during adolescence. Gender identity is an important factor to consider on long-term follow-up of OT-DSD patients.