We report a rare case of necrobiotic xanthogranuloma (NXG) of the extremities with paraproteinemia and without periorbital involvement at presentation in a 58-year-old white woman. A combination of oral cyclophosphamide and oral dexamethasone was attempted, but the patient then developed a left intraorbital lesion. Treatment was not successful in that the gammopathy did not improve and the patient continued to develop more lesions.
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