Distal myopathy with rimmed vacuoles is a rare muscle disease and, so far as we know, it has not been reported in Korea as yet. This disorder is known to be inherited as autosomal recessive trait and to have no specific treatment. Hereby, we report 3 patients of distal myopathy with rimmed vacuoles in a family. The clinical characteristics of these patients were slowly progressive symmetrical muscle weakness and wasting of all 4 extremities, worse in distal legs. The pretibial muscles were involved more markedly than the calf muscles. Serum muscle enzymes were increased. The prominent EMG findings were myopathic changes, but reduced recruitment was occasionally found in some distal muscles The muscle biopsies of right biceps brachii muscle were performed in two patients, which showed the characteristic rimmed vacuoles by light microscope. Membranous whorls and randomly oriented intracytoplasmic filaments were found by electron microscope. The severity of pathological abnormalities were related to the clinical status of the patient. One had been treated with steroid(prednisolone) for several years but with no improvement.