Necrobiotic xanthogranuloma is a rare, chronic, progressive granulomatous disorder which manifests as yellowish plaques and nodules, most commonly in the periorbital region. The exact aetiology is not known. It is commonly associated with monoclonal gammopathy. Various treatment modalities used include chemotherapy, intralesional and systemic steroids, radiotherapy and surgical excision. We report a case of necrobiotic xanthogranuloma involving the periorbital area causing cosmetic disfigurement.