XY female: Two cases with different gonads presenting as primary amenorrhea

Abstract

Two 18-year-old female patients admitted to the hospital with the complaints of primary amenorrhea. The clinical examination and investigations revealed one of them as complete androgen insensitivity syndrome and the other Swyer syndrome. Bilateral gonadectomy was performed and hormone replacement therapy started for both the patients. These two conditions should be considered in the differential diagnosis in every adolescent female patient with delayed puberty and the importance of early gonadectomy should be stressed in order to avoid the risk of gonadal tumor development.