We report three patients with keratitis, ichthyosis and deafness (KID)-syndroine. All had characteristic hyperkeratotic skin lesions and neurosensory hearing defects. Two had ophthal-mologic symptoms. The third patient did not have eye involvement at the age of 3 years, but exhibited the other typical signs of the syndrome. In none of the three cases were any of the patients’relatives affected, and a spontaneous new mutation is the most likely explanation for the occurrence of this rare syndrome. Histopathological and electron microscopic studies revealed orthohyperkeratosis but no other pathology and no abnormal deposits of glycogen were found. Treatment with the aromatic retinoid etretinate proved to be of little value in any of the patients. The necessity for early audiologic and ophthalmologic evaluation and the need for lifelong medical care for patients with KID-syndrome is emphasized.