Necrobiotic xanthogranuloma (NXG) is a rare multisystemic disease that manifests as cutaneous inflammatory lesions and has a strong association with paraproteinemia and an increased risk of plasma cell dyscrasias and other lymphoproliferative disorders. Its general skin manifestation is multiple, asymptomatic, indurated nodules or plaques with a yellowish hue which frequently affect the periorbital regions. This case is unusual in that cutaneous lesions developed only at the extremities without periorbital involvement, accompanied by multiple myeloma.