Dysplasia epiphysealis hemimelica of the left proximal femur was diagnosed in an eight-month-old girl. At the age of 18 months, radiographs of the hip and MRI showed overgrowth and loss of containment of the femoral head. She underwent resection of the superior portion of the head and neck of the femur at the age of 2.5 years. Six months later further radiographs and an MR scan show that the mass has increased in size and that hip containment has been lost. Further plain radiographs have shown that the left knee, ankle and spine were involved.

To the authors’ knowledge, this is the first report of dysplasia epiphysealis hemimelica involving both the lower limb and the spine. A review of the literature is presented.