An unusual duplication of the hypophysis is described in a female baby aged 26 days with a median cleft face syndrome. Malformations of the cranium, brain, and spinal cord were confined to the ventral midline. These findings contrast to dysraphism, in which anomalies of dorsal neural and/or mesodermal structures are common. This rare condition of double hypophysis should also be classified among the midline cleft face syndrome in contrast to the formerly recorded cases of double hypophysis in partial twinning.