Necrobiotic xanthogranuloma (NXG) is a rare inflammatory histiocytic disease of the skin. Xanthogranuloma of the central nervous system is rare and few cases have been reported. To the authors' knowledge, there has been no previously reported case of NXG in which an intracranial lesion was found. This 52-year-old man, in whom NXG with all its cutaneous manifestations had been diagnosed, presented with three episodes of generalized tonic-clonic seizures. A contrast-enhanced computerized tomography scan of his brain revealed a bifrontal, dura-based mass lesion. The lesion was excised and reported to be an NXG that was similar, but not identical to the skin lesions. The patient was placed on a regimen of antiepileptic drug and chlorambucil after surgery.