Dysplasia epiphysealis hemimelica was defined by Trevor (1950) as a rare congenital growth disorder of the tarsus and of the epiphysis of the long bone. In this report, a rare case of dysplasia epiphysealis hemimelica associated with multiple extraskeletal osteochondromas is presented. Although different modes of expression of the same pathologic process have been suggested for dysplasia epiphysealis hemimelica and osteochondroma, the biological feature of cartilaginous overgrowth in the skeletal system still seems unclear.