Ethmocephaly is the rarest form of holoprosencephaly, which occurs due to an 
incomplete cleavage of the forebrain. Clinically, the disease presents with a 
proboscis, hypotelorism, microphthalmos and malformed ears. Amniotic band 
syndrome is another rare congenital malformation with ring-like constriction 
bands in the limbs, head, face or trunk. We present a case of ethmocephaly with 
amniotic band syndrome, which is likely the first of its kind, published in the 
literature.