Carpal-tarsal osteolysis is a rare disorder of unknown etiology, Gradual resorption of the carpus and the tarsus begins in early childhood and progresses during the period of growth resulting in severe deformities and disability in adult life. A 13-year-old Korean girl with striking carpal and tarsal osteolysis is reported. This patient had no evidence of hereditary transmission, nor of nephropathy at the time of writing.