Trevor disease or dysplasia epiphysealis hemimelica (DEH) is an extremely rare condition with incidence of about 1:1,000,000. Male to female ratio of reporting case is 3:1, and usually diagnosed between two and eight years old. It usually affects the medial portion of the joint, but lateral involvement is not uncommon. Hip-joint was affected in less than 4% of existing cases in the literature. It would be very important to precisely mange the hip involvement to prevent from further articular cartilage destruction in this very young age.
We report an infant boy with isolated DEH of hip. We found a total of 271 cases of DEH that reported between 1926 and 2017.The most sites of involvement are ankle joint and around the knee. Our search reaches out to ten cases of hip involvement. Hip involvement needs a patient specified decision. We observed our patient for three years with a desirable hip joint function.