Four patients with congenital ichthyosiform hyperkeratosis are presented. One patient had generalized hyperkeratotic lesions associated with bullous episodes. Two patients had systematized hyperkeratotic lesions without bullous episodes. The fourth patient had a localized hyperkeratotic lesion without bullous episodes. Biopsies from lesions on each of the four patients showed similar edematous and dyskeratotic changes in the epidermis of the type described for bullous ichthyosiform erythroderma. Because of the striking histologic similarities of lesions in these four patients, it is concluded that localized variants of bullous congenital ichthyosiform erythroderma occur and present themselves clinically as epithelial nevi.