Familial hyperlipoproteinemia associated with intracranial xanthoma belonged to a very rare entity and the authors were able to collect only one reported case in our domestic literature. A 57-year-old house wife was admitted with a six months' history of progressive hearing impairment on the left and some cerebellar signs characterized by ataxic gait. Physical examination revealed several hard and painful subcutaneous masses around the joints of four extremities, which had enlarged slowly within the last three years. Also, remarkable deformity of Achilles tendon was seen on both sides. Neurological examination showed bilateral papilledema, left hemifacial hyp- and dysesthesia, left hearing difficulty and left cerebellar signs. Laboratory examination reported markedly elevated serum cholesterol level as high as 575 mg/dl, and determination of the serum lipoprotein disclosed the findings compatible with Type II a hyperlipoproteinemia. Plain skull X-rays showed osteolytic defect of the left occipital bone and CT scan demonstrated a large irregular low density mass extending into the left posterior fossa, which showed ring like enhancement and spotty high density of calcification. Angiogram suggested the mass was of less vascular lesion and situated extramedullary. Suboccipital craniectomy was performed and an epidural solid mass was resected, although the mass was markedly growing into the posterior cranial cavity. Microscopic examination showed the findings typical to the xanthoma, which was totally coincided with that of biopsy specimen obtained from the tumor over the ankle. Electron microscopic examination was performed and various kinds of lipid inclusions in the cytoplasma of xanthoma cells, such as dense bodies, volute or onion-like figures and lucent large vacuoles, were observed.