A case report of a 12-year-old child with a spinal extradural angiolipoma is presented. The tumor was totally removed and a good recovery was obtained. In a review of 43 previous cases, the clinical, radiological, and histological features of the tumor are discussed. It is stressed that spinal angiolipomas and spinal lipomas have to be considered as two different clinicopathological entities in order to ensure adequate treatment and prognosis.