Abstract
Leukocyte adhesion deficiency type I is an extremely serious and rare form of congenital immunodeficiency with recurrent episodes of infection since neonatal period. It is usually diagnosed in childhood, but if diagnosed early in neonatal period then definitive treatment can be instituted early with promising results. High index of suspicion is necessary to diagnose this condition in neonates. Neonate may present with serious bacterial infections with a leukemoid reaction.