Collodion baby is a rare keratinizing congenital disorder. Although it is milder in degree than harlequin fetus, the infant is at risk for increased water loss, thermal instability, percutaneous toxicity, and infection as a result of an impaired skin barrier function. Here we report on an 11 days-old collodion baby with hypernatremic dehydratation, septicemia and congenital hypothyroidism. To our knowledge congenital hypothyroidism associated with collodion baby is reported in only one case up to date.