Dysplasia epiphysealis hemimelica (DEH), also known as Trevor’s disease, is a rare disease which is skeletal developmental disorder characterized by asymmetrical growth of epiphyseal cartilage. It most frequently affects the ankle joint. Surgical treatment with complete resection is recommended before irreversible joint damage and deformity occurs. Multiple surgeries may be required if resected at an early age because recurrences are common until skeletal maturity. We present a case of DEH at the anteromedial aspect of a left ankle joint and a review of the literature.