Gorham's disease is a rare condition of unknown etiology, which is characterized by intra-osseous vascular abnormalities leading to osteolysis and sometimes, extension to adjacent tissues. Although there is no histological evidence of malignancy, the lesions display an aggressive potential responsible of osteolysis with pathological fractures and respiratory failure. The extension of the disease varies from one location to multiple bone lesions throughout the skeleton and the clinical presentations depend on the sites of involvement. In the ISSVA (International Society for the Study of Vascular Abnormalities) classification, its affiliation to the group of vascular tumors or vascular malformations is still debated. To date, there is no standard treatment defined for this disease. The proposed treatments are inhibitors of bone resorption (bisphosphonates) and interferon alpha therapy or anti-angiogenic molecules. Radiation therapy and surgery may also have a place in the therapeutic strategy.