Dysplasia Epiphysealis Hemimelica (DEH)is a rare developmental epiphyseal disease characterized by asymmetri cover growth of cartilage in the epiphysis, histologically mimics osteochondroma of the joints. The disease mainly involves ankle, knee and tarsal bones. Small number of cases is reported in literature particularly those managed arthroscopically. The present study reports a case of Dysplasia Epiphysealis Hemimelica (DEH) developed in the talus in male child presented with swelling, pain, and inability to put weight. Radiographs showed irregularities of the talar body with a large loose body in the anterior joint compartment with excessive overgrowth of the anteromedial and posteromedial aspects of the talus managed by combined anterior and posterior ankle arthroscopy in a single session and showed improvement of both pain and range of motion in the short-term follow-up. Trevor disease of the ankle is a rare disease that needs high index of suspicion for diagnosis and arthroscopy can be a minimally invasive option to improve pain and range of motion. This case report demonstrates basis on which such a rare disease can be diagnosed and shows the arthroscopic surgical technique details and its possible value in the outcome of these cases.