Dysplasia epiphysealis hemimelica (DEH), known as Trevor's disease, tarsoepiphyseal aclasis or la tarsomegalie, is a rare disease characterized by cartilaginous overgrowth of one-half of a single limb. Until now only three cases have been reported in Korea. The ages at initial diagnosis were 2.3 years, 2.5 years and 11.5 years, and patients were two boys and one girl by sex. The chief complaints were genu valgum (2 cases) and valgus of the foot (1 case). Plain radiographs showed bony overgrowth and asymmetrical ossification centers in all cases. MRI showed a definite cartilaginous or osteocartilaginous lesion (T1-weighted: intermediate signal intensity (SI) and T2-weighted: intermediate SI mixed inner high SI). Arthroscopic removal of the loose body was performed in one case and spontaneous regression of the genu valgum was noted in one case. We report upon three additional cases and review the literature.