We report a case of alobar holoprosencephaly (HPE) and cebocephaly associated 
with uncontrolled maternal type 1 (insulin-dependent) diabetes mellitus. Alobar 
HPE is the most severe form of HPE. Patients with cebocephaly have ocular 
hypotelorism and a proboscis with a single, blind-ended nostril. Shortly after 
our patient was born, we were consulted for airway management, as the parents' 
goal was to bring their child home. A tracheostomy tube was placed, and choanal 
atresia repair was eventually performed. The infant was never decannulated, 
however, and she died at the age of 9 months of acute respiratory distress 
syndrome secondary to an upper respiratory infection. To the best of our 
knowledge, this case represents the longest reported survival of an infant with 
alobar HPE and cebocephaly. Decisions regarding the care of these infants should 
be made in a collaborative, multidisciplinary fashion, with special attention 
paid to the primary caregivers' goals of care.