Necrobiotic xanthogranuloma is a rare non-Langerhans-cell histiocytosis. A 62-year-old woman presented with yellowish erythematous plaques, nodules, and papules in the periorbital region and the extremities. She had a nodular tumor grown on the left upper lid that clinically resembled a keratoacanthoma. Histologically it was a xanthogranulomatous lesion. She suffered from monoclonal gammopathy of unknown significance of κ‑type. Treatment was realized with a combination of systemic dapsone/prednisolone and topical corticosteroids.