Angiolipomas are neoplasms composed of mature adipocytes admixed with abnormal vascular elements. They are most commonly found in the subcutaneous tissue of the trunk and extremities, but other sites have been reported. The craniospinal axis is an uncommon but significant site. An extensive review of the literature is conducted. We summarize 94 cases of angiolipomas in the central nervous system (CNS) in 92 patients, including five in our own series, to highlight the most prominent features of these tumors. The increasing number of cases of CNS angiolipoma in the era of magnetic resonance imaging raises the question of the rarity of these lesions.