A single nostril associated with alobar holoprosencephaly is a rare co
ngenital lesion. This paper reports two female term neonates with holo
prosencephaly. The first neonate asphyxiated at birth had a single nos
tril, hypotelorism, posterior cleft palate, inferonasal coloboma of th
e iris and disc and persistent tunica vasculosa lentis. The other neon
ate had cleft lip and palate and recurrent convulsions. Both neonates
had gross motor and developmental delay. Cranial sonography and CT sca
n showed features consistent with alobar holoprosencephaly. Karyotypin
g for the first neonate and her family members was normal. Both of the
m were treated conservatively and supervised.