Spondyloepiphyseal dysplasia tarda (SEDT) is a heterogeneous group of
bane dysplasias. A clinical and radiographic study is presented of two
patients, members of the same family, with the autosomal recessive fo
rm of SEDT. Radiographs of the vertebrae showed the characteristic axe
-like configuration. Premature coxarthrosis developed in the 4th decad
e and progressed slowly over 20 years.