We present a rare case of a femoral shaft fracture in a 74-year-old woman with a preexisting untreated bilateral congenital hip dislocation and with concomitant leg length discrepancy. Because of the marked deformity of the upper femur, a derivative of the congenital hip disease, an open reduction and internal fixation was performed, with the use of an anatomic femur plate originally designed for the treatment of periprosthetic fractures. The patient was treated successfully and returned to her previous status of activity. The management of a femoral fracture in the presence of ipsilateral hip disease is a challenging issue. The surgical treatment choice in such cases has to be individualized, taking into consideration several anatomic and medical parameters.