The KID syndrome is characterized by congenital ichthyosis, vascular keratitis and neurosensorial deafness. We report a 17 year old female patient, the first case of KID syndrome in Spanish literature. Red, dry, scaling skin was present at birth with sparse hair. At the age of six, malar erythema was prominent, with perioral ragades and onset of progressive neurosensory deafness. At the age of ten, vascularizing keratitis developed. At 12, treatment with etretinate failed to improve the ichthyosis. We review the clinical, pathological and analytical features of KID syndrome and discuss its relationship to other ichthyoses.