Netherton's syndrome is characterized by ichthyosiform desquamation, bamboo hair and often atopic diathesis. It is transmitted as an autosomal-recessive trait. In this paper we report a baby with Netherton's syndrome who developed hypernatremia during the neonatal period. This complication should be remembered in erythrodermic infants as a preventable cause of neonatal morbidity.

Keywords: Netherton's syndrome, newborn, hypernatremia