Abstract:
Persistent Mullerian Duct Syndrome (PMDS), a rare
form of male pseudohermaphroditism. It is
characterized by the persistence of Mullerian duct
structures (uterus, fallopian tubes and upper two-thirds
of vagina) in otherwise normally virilized males
(Karyotype 46XY). The exact cause of PMDS is not
known, however it is thought to result from the defect
of the synthesis or release of Mullerian inhibiting
factor (MIF) or from a MIF receptor defect. Herein we
report a case of PMDS with gynaecomastia and
hypospadias in a post orchidopexy patient.