Background & objectives: Ovotesticular disorder is defined as thepresence of both ovaries and testes in the same person, regardless ofkaryotype. Patients with ovotesticular disorder have a higher risk ofdeveloping gonadal neoplasms such as gonadoblastoma or seminomathan the general population.Methods: The karyotype analysis of a 32-year-old male patientwith phenotype who was examined for bilateral abdominal undescendedtestis was 46 XY. In his radiological examination, acomplex internal genital structure compatible with ovotestis wasdetected. The patient whose operation material was examined inour unit is presented with histopathological and clinical features.Results: Macroscopically, the specimen consisted of bilobed gonadalstructures and a single cordial structure adjacent to it. In the histopathologicalexamination, in addition to seminoma, tissues including vesicleseminalis, atrophic testis, tuba uterina, endometrium and endocervixcomponent were seen. Most of the structures thought to belong to thefemale genital tract were not of the usual morphology, possibly due toinsufficient hormonal effect. Immunohistochemical markers contributedgreatly to the distinction of the organs and diagnosis. The patient,who was diagnosed with ovotestis and Stage I seminoma based onhistopathological findings, received a single dose of carboplatin chemotherapyand free of disease at 6th month of follow-up.Conclusion: Ovostesticular disorder is a very rare sex developmentaldisorder, with a 10% risk of germ cell tumour developmentin 46XY and mosaicism cases.As a result, orchiectomy is required in the presence of an undescendedtestis or development of a tumour. Because male andfemale genital organs have similar morphological and immunohistochemicalfeatures, a multidisciplinary approach is requiredfor pathological examination.</p
