A 61-year-old Japanese man was treated for eosinophilic granuloma of the soft tissue in the orbit. The clinical characteristics were typical for eosinophilic granuloma of the soft tissue: 8% eosinophilis, very soft tumor masses in both orbits, multifocal lesions including the salivary glands, good response to steroid hormone, and hypergammaglobulinemia. The ocular signs were exophthalmos and lid swelling. Light microscopy showed many lymphoid follicles with many eosinophils, also in other parts of the body. The previously reported cases of orbital lesion are reviewed. Patients with orbital eosinophilic granuloma of the soft tissue are apt to be 30–50 years of age, older than patients without ocular lesions.

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