We present a case of generalized congenital ichthyosiform dermatosis associated with neurosensory hearing loss, neovascularization of corneas, partial alopecia and abnormalities of nails and teeth on a 26 years old female patient. This case is strictly similar to the previously reported cases. The combination of these abnormalities was stuggested to be a new syndrome by some authors. This case is the first reported case in Korea, although in the literature we could find several cascs with similar patterns of malformations.