A case is described of subdural spinal lipoma with posterior fossa extension and the world literature is reviewed. A high proportion of high cervical lipomas extend into the posterior cranial fossa. Many cases were probably missed in the pre-computed tomography era. Those cases with posterior fossa growth are more likely to be found in infants or those cases with symptoms dating from birth; most present with quadriparesis. All cases of high cervical lipoma demonstrated by myelography should be submitted to brain computed tomography in order to exclude posterior fossa extension and demonstrate the presence of hydrocephalus.