Netherton syndrome (NS) is an autosomal recessive disorder characterized by congenital ichthyosis, trichorrhexis invaginata, and severe atopy.1 It presents in the neonatal period, and the ichthyosis develops into serpiginous plaques, bordered by a double-edged scale, termed ichthyosis linearis circumflexa. Hair shaft abnormalities present later in life.1,2 We report a case of NS successfully treated with dupilumab, an interleukin (IL) 4 and IL-13 α antagonist.