Dysplasia epiphysealis hemimelica (DEH) or Trevor's disease is a rare disorder affecting the epiphyses and short bones of the limbs, and characterized by a benign overgrowth of the medial-half of the epiphysis resembling osteochondroma. Upper limb involvement is very rare and most commonly affects the carpal bones. Only five established cases of DEH around the elbow were found in the orthopedic literature. We herein report an additional case of Trevor's disease of the elbow in a child with a symptomatic ulnar nerve compression and its management. To the best of our knowledge, this is the first case of DEH of the elbow presenting with nerve compromise.