Olmsted syndrome is characterised by mutilating palmoplantar keratoderma with peri-orificial hyperkeratosis. We report the case of an 8-year old boy who presented with severe keratoderma of the soles since birth and of the palms from the age of 3 years. At 3 years of age hyperkeratotic plaques appeared on the elbows and knees. The child developed keratotic lesions at the angle of the mouth 1 year later. The child had sparse thin easily pluckable hair. Light and scanning electron microscopic examination of the hair revealed several hair shaft abnormalities. Though the psychomotor development of the child was normal till 1 year of age, thereafter the keratoderma had largely restricted the child's mobility. There was no history of hyperhidrosis and no dental abnormality was detected. The lesions had been unresponsive to keratolytics and had recurred after surgical removal. The patient was started on oral retinoids and topical keratolytics and had partially responded in 2 months.