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Necrobiotic xanthogranuloma (NXG) is a rare histiocytic disease which most frequently involves periorbital areas. NXG is associated with paraproteinemia with a ratio over 80%. However, a few cases of NXG without paraproteinemia (isolated NXG) have also been reported. Main observation: A 58-year-old Japanese woman complained about asymptomatic dermal nodules on the bilateral lower eyelids. The histopathological examination revealed granulomatous inflammation. Paraproteinemia was not detecteddoi:10.7241/ourd.20133.84 fatcat:wfxpqspitnh7zbxsfkkh3opn3m