Seven cases of A. T. D. are presented. Radiological findings were extraordinarily diverse. The prognosis of the disease is difficult for each individual case, because of the frequent pulmonary complications and cystic renal lesions are not always directly related to the severity of the bone changes. The authors believe that the term "thoracic-pelvic-phalangeal dysplasia" proposed by Langer, is the most adequate, since it points to the key factors in the diagnosis of the disease.