We report a case of KID syndrome in a young girl born to non-consanguinous parents without any similar family history. The typical features of this dysplasia, erythrokeratodermia with dry rugous teguments, pachydermia folds of the knees, facial erythema, peribuccal grooves, leucokeratosic perleche, hypotrichosis of the eye lashes and eyebrows, early onset deafness and ophtalmological lesions were observed. The characteristic pachydermatoglyphia of the hands was present. A malformation of the posterior cerebral fossa--a dandy walker syndrome--was revealed at computed tomography.