Dysplasia epiphysealis hemimelica, also termed Trevor disease, is a rare disorder that, although benign in nature, can be locally aggressive, particularly when affecting the ankle joint, which is the joint most frequently affected, followed by the knee. The female:male ratio is 1:3, and it is generally diagnosed between 2 and 14 years of age. Surgical treatment with complete resection is recommended before irreversible joint damage and deformity occurs. We presented a case in which dysplasia epiphysealis hemimelica was diagnosed on the medial aspect of a right ankle joint.