Persistent Müllerian Duct Syndrome (PMDS) is a rare condition in which Müllerian duct structures, i.e. the uterus, cervix, fallopian tubes and upper two-thirds of the vagina, persist in a phenotypically and karyotypically male patient. It is caused either by an insufficient amount of Mullerian inhibiting factor (MIF) or due to insensitivity of the target organ to MIF.