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Dysplasia epiphysialis hemimelica (DEH), also known as Trevor disease, is a very rare disease characterized by an overgrowth of the osteocartilaginous epiphyseal caused by unknown factors. The medial side of the epiphysis is affected twice as often as the lateral side and more than one epiphysis was affected in two-thirds of the cases. Only a few cases of DEH in the upper extremities have been published, as the lower limb is usually the most affected, especially in the bones of the ankle joint. The symptoms vary from little significant deformities to symptomatic cases, pain, and decreased function depending on the location and the size of the lesion. When surgery is indicated, the complete excision of the lesion is the procedure of choice. If this is not possible, because of the location or size of the lesion, a realignment osteotomy may be necessary. We report the case of a 12-year-old patient with a distal epiphysis DEH on the left radius, treated by wedge osteotomy realignment at the level of the lesion with Kirschner wires. The clinical outcome of realignment osteotomy of the radial epiphysis has been the correction of the deformity with a normal and painless function of the wrist and hand. The case reported was an extremely rare location in the distal radial epiphysis of DEH. We are aware of only one case published previously with involvement of the distal radius in children. The surgical treatment of realignment osteotomy without complete removal of the lesion has enabled anatomical and functional improvement of the joint as well as a reduction in lesion size., Level of Evidence: Level IV case report.