Gorham's disease is a rare condition of bones characterized by spontaneous massive and progressive osteolysis. Less than 200 cases have been reported so far. Femur is an uncommon site of Gorham disease. We report a young female, presenting as pathological fracture of the femur, wherein rapid osteolysis of femur occurred. The clinical, pathological, and radiological picture suggested a diagnosis of Gorham's disease. The patient was subjected to radiotherapy (40 Gys) and put on bisphosphonates. At a follow-up of 2 years, the disease process had stopped and partial recalcification of the bone had occurred.