Olmsted syndrome is a rare keratinisation disorder characterised by mutilating palmoplantar keratoderma with peri-orificial hyperkeratosis. Till now around 73 cases have been reported. We are reporting a case of 3 year old female child with keratoderma of palms and soles since birth along with peri-orificial hyperkeratosis flexion deformity of digits of palms hypotrichosis and onychodystrophy. There was no history of hyperhidrosis no dental abnormalities and no sensorineural hearing loss. Hence the diagnosis of Olmsted syndrome was made.