Necrobiotic xanthogranuloma is a progressive histiocytic granulomatous disease with frequent extracutaneous involvement. We have documented an unusual case of necrobiotic xanthogranuloma in a 58-year-old man with a history of recalcitrant and chronic arm and leg ulcerations in the absence of characteristic periorbital involvement. Our patient demonstrates a therapeutic challenge and may display a new necrobiotic inflammatory disorder not previously characterized.