The authors describe a case of a 42-year-old woman with systemic xanthogranulomatosis who primarily manifested bilateral intraorbital tumors, generally effecting many regions. These included the intracranial dura mater, the spinal cord, the retroperitoneum, the pericardium, and the mediastinum. This disorder is basically similar to systemic Weber-Christian disease except for the absence of subcutaneous panniculitis. Immunohistochemical study reveals that this clinical entity is different from histiocytosis X because the origin of the foamy cells is not the T-zone histiocyte but the macrophage. The treatment of this disease is also discussed.