Necrobiotic xanthogranuloma (NXG) is a rare dermatosis with a poorly understood pathophysiology. Studies comparing treatments for such lesions are limited. We present the case of a patient with a 30-year history of NXG refractory to several individual therapeutic interventions [excision, intravenous immunoglobulin (IVIg), systemic chemotherapies and immunosuppressants, cryotherapy and laser therapy], who ultimately responded to a combination of treatment with electron beam radiation therapy (EBRT) in conjunction with IVIg. This combined treatment resulted in flattening of the NXG lesions and a reduction of symptomatic pruritus within the treatment zone. EBRT may represent a potent treatment for NXG, and formal trials evaluating its effectiveness may yield insights into the management of NXG.