A case of mutilating palmoplantar keratoderma is reported.A 21-year-old female presented hyperkeratosis of the palms and soles for 11 years,constriction bands in the distal interphalangeal joints of the hands for half a month,ulceration on the extensor surface of the distal interphalangeal joints of the middle finger of the left hand for 4 days.Histopathologic examination of the lesion on the right hand showed hyperkeratosis,thickening of granular layer,acanthosis,elongation of the rete pegs,perivascular infiltration of lymphocytes in the superficial dermis.The patient was diagnosed as mutilating palmoplantar keratoderma.