Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome (or Hay-Wells syndrome) is a rare congenital
malformation. Our first cases were a pair of female monozygotic twins with AEC syndrome at Srinagarind Hospital. In this
study, we describe monozygotic female twins concordant for ankyloblepharon, ectodermal dysplasia and helical rim deformities,
but discordant for cleft, syndactyly of toes, heart and urinary tract abnormalities. Twin A had syndactyly of the right third and
fourth toes with incomplete bilateral cleft lip and complete bilateral cleft palate. Twin B had left ventricular enlargement,
caliectasia of both kidneys with complete left unilateral cleft lip and cleft palate. The twins were treated by multidisciplinary
teams with satisfactory results.

Keywords: Ankyloblepharon-ectodermal defects-cleft lip/palate syndrome, AEC syndrome, Hay-Wells syndrome, Monozygotic
twins