Keratitis-ichthyosis-deafness (KID) syndrome is an autosomal recessive syndrome of ectodermal dysfunction caused by a mutation of the connexin 26 gene on chromosome 13. KID syndrome is characterized by progressive inflammation and thickening of the cornea (vascularizing keratitis), which may not present until later childhood. Infants present at birth or soon after with mild diffuse erythema and fine scale (often diagnosed as ichthyosiform erythroderma). This resolves, but, within the first year and generally within 3 months, the predominant erythrohyperkeratotic lesions appear, which are sharply defined, irregularly shaped hyperkeratotic, verrucous plaques with scale predominantly on the face and limbs, thickening of the skin of the palms and soles, and hyperkeratotic plaques on the knees, elbows, and dorsum of the hands and feet. Patients look prematurely aged. The hair is sparse or absent and difficult to control; the nails are small and malformed. There is a congenital nonprogressive neurosensory hearing loss. Bacterial, fungal, viral, and mite infections of the skin are troublesome in these patients.