Gorham disease (GD) is a rare osteolysis without sex, race, or age predilection, affecting bones in different regions. Based on clinical, histological, and molecular features, diagnosis is difficult and required exclusion of neoplastic, inflammatory, infectious, and endocrinologic disease. Etiology is still unknown. We report the case of a 36-year-old man suffering from severe progressive osteolysis located at the mandible. Histology showed massive osteolysis without malignant cells. Immunohistochemistry revealed thin-walled vessels and lymphatic ducts. These investigations lead to diagnosis of GD. Radical surgical treatment was followed by bisphophonate therapy. Recurrence occurred 4 months after surgery and alphaa-interferon therapy permitted remission. To support this case report, we reviewed the 41 maxillofacial cases published in the literature since 1928. Jaw is the main location; histology mostly shows hemangioma-like proliferation. Immune disorders are usually advanced as a cause although physiopathology is unknown. Therefore, appropriate treatment is controversial. Antiosteoclastic drugs are usually proposed in addition to surgery, but immunomodulating drugs and radiation therapy should also be considered in the treatment.