Dysplasia epiphysealis hemimelica (DEH) or Trevor's disease is a rare, nonhereditary developmental disorder of skeleton affecting epiphysis and short bones of limbs and characterized by a benign overgrowth of the medial half of the epiphysis resembling osteochondroma. We herein report an unconventional presentation of Trevor's disease of the hip with the involvement of the whole epiphysis. Only a few cases of DEH with such unusual features were found in the literature. The aim of this case report is to spread the awareness among the doctors about an unusual case of DEH with the involvement of the whole capital femoral epiphysis which has been neglected for 10 years. We also discuss the natural history of the development of disease, challenges faced during the course of treatment, clinical results, and complications.