Gorham's massive osteolysis is one of the five classical types of idiopathic osteolysis. The femoral localisation is rare. The diagnosis is based on anamnestic data (non-hereditary), on biochemical data (absence of nephropathy), on radiographical data (progressive monocentric osteolysis without periosteal reaction), and on histological data (intraosseous angiomatosis with either capillaries or lymph vessels, or both; eventually fibrosis). Nowadays, treatment mostly consists of amputation or arthroplasty, combined with radiotherapy. Spontaneous arrest of the disease occasionally occurs, but this is unpredictable. The possible role of gene-therapy in the regulation of osteoclastic activity has to be determined in the future. Review of the literature produced 22 cases of Gorham's massive osteolysis with a localisation at the femur massive osteolysis, including one personal case.