Ovotesticular disorder of sex development (true hermaphroditism) is a rare form of sex development disorder, accounting for less than 10% of all cases. This condition is characterized by the simultaneous presence of both ovarian and testicular tissue, regardless of karyotype. This article presents a clinical case of a 61-year-old female patient diagnosed with a disorder of sex development for the first time. During a routine outpatient ultrasound examination, the ovaries appeared enlarged with active blood flow, which was disproportionate to the patient’s age and postmenopausal status. The uterus and cervix were absent. The patient reported primary amenorrhea, no history of pregnancies, and an active sexual life since the age of 20. She had been married since the age of 25 and had never sought medical advice regarding infertility or amenorrhea. Further genetic and cytogenetic analysis revealed a 46,XY karyotype, associated with a high risk of gonadal malignancy. Bilateral adnexectomy was performed. Histological examination confirmed the presence of both ovarian and testicular tissue in the gonads, consistent with a diagnosis of ovotesticular disorder of sex development with bilateral ovotestes. In cases with a 46,XY karyotype, bilateral adnexectomy is indicated due to the significant risk of malignancy.

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