RESUMEN
Proximal focal femoral deficiency (PFFD) is a developmental disorder of the proximal segment of the femur and of acetabulum resulting in shortening of the affected limb and impairment of the function. It is a spectrum of congenital osseous anomalies characterized by a deficiency in the structure of the proximal femur. The diagnosis is often made by radiological evaluation which includes identification and description of PFFD and evaluation of associated limb anomalies by plain radiographs. Contrast arthrography or Magnetic Resonance Imaging is indicated when radiological features are questionable and to disclose the presence and location of the femoral head and any cartilagenous anlage. The disorder is more commonly unilateral and is apparent at birth. However, bilateral involvement is rarely seen. Therapy of the disorder is directed towards satisfactory ambulation and specific treatment depending on the severity of dysplasia.