Dysplasia epiphysealis hemimelica is a rare developmental disorder which affects the epiphyses. We report a case of the disease located in the ankle joint, referred to our clinic with the initial misdiagnosis of a Salter-Harris 3 type fracture of the distal epiphysis of the tibia. After correct diagnosis, the patient was treated surgically with the excision of the cartilaginous masses. Taking an accurate medical history and performing adequate imaging studies is essential in diagnosing and treating this disease. Fracture-like epiphyseal configurations in patients with no history of injury should raise suspicion of periarticular developmental disorders.