Dysplasia epiphysealis hemimelica is an uncommon disorder primarily involving the epiphyes of the lower extremities. We report imaging findings of a case of dysplasia epiphysealis hemimelica involving the trochlear epiphysis of the humerus. Conventional radiography showed irregular ossification in the area of trochlear epiphysis, the finding thought to be diagnostic of this discorder. However, other imaging modalities, such as double contrast arthrography, computed tomography and MR imaging, provided valuable information about the nature and extent of the epiphyseal pathology