A brief report of persistent mullerian duct syndrome (PMDS) with 46XY karyotype which is one of the rarest variety of disorders of sexual differentiation (DSD) accounting only 5% cases of all is being presented. A 21 years old male with left inguinal hernia and absent right testis presented in surgical outdoor and was operated. On exploration female genital organs like uterus and fallopian tubes along with contralateral testis were present in left inguinal canal as a content of sliding left inguinal hernia.
Key words: Mullerian duct syndrome, karyotype, disorders of sexual differentiation (DSD), cryptorchidism, inguinal hernia.
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