Necrobiotic xanthogranuloma is a very rare, histiocytic disease characterized by destructive skin lesions and often arising in the setting of monoclonal gammopathy. A diagnosis of necrobiotic xanthogranuloma is made based on its typical cutaneous features and therefore requires experienced physicians who are familiar with its appearance. Here, we present a patient with highly distinctive cutaneous lesions of necrobiotic xanthogranuloma occurring in chronic lymphocytic leukaemia.