Abstract: Necrobiotic xanthogranuloma (NXG) is a rare chronic progressive multiple organ disease, and it is a subtype of adults’ yellow orbital granuloma. The typical clinical manifestations of NXG are multiple sclerosing yellow-brown plaques or nodules around the orbit, and the lesions may have atrophy, ulcer and telangiectasia. NXG mainly affects the skin and subcutaneous tissues. It can also affect other body systems and internal organs, also causing serious complications of eye. At present, the etiology and pathogenesis of NXG are unknown. The most prominent histopathological features of NXG is large areas of denatured collagen with granulomatous inflammation, and cholesterol crystal Touton giant cell, foam cells and foreign body giant cell can be seen in the lesion specimen. The prognosis of NXG is usually good, but there is no radical treatment. Chemotherapy with alkylating agents is most commonly used, and corticosteroids combined with chemotherapy can be used to treat NXG with other systemic diseases. Focal glucocorticoid injection, radiotherapy, and surgical excision may be used for local and minor lesions. Other treatments include interferon, plasmapheresis, and intravenous immunoglobulin and so on, but the results vary. (Int Rev Ophthalmol, 2021, 45: 165-171)

Key words: necrobiotic xanthogranuloma/ diagnosis, necrobiotic xanthogranuloma /treatment, orbit ,