True hermaphrodite is one of the rarest variety of disorders of sexual differentiation (DSD) and represents only 5% cases of all. A 3-year-old child presented with left sided undescended testis and penoscrotal hypospadias. Chordee correction was performed 18 months back, elsewhere. At laparotomy Mullerian structures were present on left side. On right side testis was normally descended into the scrotum.
Keywords: Disorders of sexual differentiation; Persistent mullerian duct syndrome; True hermaphrodite.