Necrobiotic xanthogranuloma (NXG) with paraproteinemia is a rare non-x histiocytosis with conspicuous lesions mainly located on the periorbital skin. A 68-year-old woman, with a previous history of lymphoplasmacytic lymphoma associated with IgG monoclonal gammopathy over a period of almost 4 years, presented typical lesions of NXG on the periorbital regions and left buttock. Treatment with dioxide carbon laser resulted in great improvement of cutaneous lesions, and no evidence of relapse after a 12-month follow-up. The association of NXG with lymphoplasmacytic lymphoma has not been previously described to our knowledge. The treatment of these lesions represents a true challenge for the clinician and palliative treatment of cutaneous lesions of NXG with CO(2) laser may constitute an alternative treatment in selected cases.