Progressive osteolysis with disappearance of the carpal and tarsal bones occurs as a rare syndrome associated with a serious nephropathy. The characteristic skeletal changes have an onset in early childhood and precede renal failure by 10--15 years. The present case, the ninth reported in the world literature, was a 22-year-old, white female who presented with carpal and tarsal swelling at age 2. Severe hypertension and azotemia were present when the patient died of pneumonia and extracellular fluid volume depletion. At necropsy, the kidneys weighed 55 g each. Microscopy showed proliferation of vascular intima and medial hypertrophy of renal vessels of all sizes. Tubulointerstitial and glomerular changes are probably secondary to the severe vascular disease. Although similar skeletal lesions can be seen in kindred with autosomal dominant and recessive inheritance, all reported cases of nephropathy including the present one are sporadic.