Hystrix-like ichthyosis with deafness (HID) syndrome represents a distinct disorder of keratinization that occurs in both sexes. Bilateral neurosensory deafness and hystrix-like ichthyotic erythroderma are present in infancy. far, all reported cases have been sporadic. The first patient affected with HID syndrome was described in 1977 and because of peculiar ultrastructural features the disease was initially named “ichthyosis hystrix gravior, type Rheydt” after the city of origin of this patient. Twenty years later, the same patient presented in our department with his son born in February 1997. The 3-month-boy has neurosensory deafness and typical cutaneous involvement including red hyperkeratotic papules on the face and disseminated induration of entire skin with diffuse dark-yellow hyperkeratoses. A skin biopsy has so not been permitted by the parents. This is the first report of familial occurrence of HID syndrome. Documentation of father-to-son transmission provides evidence of autosomal dominant inheritance of the trait.