We report an unusual cause of leptomeningeal MR enhancement, amyloid, along the surfaces of the spinal cord and brain stem and in the spinal subarachnoid space, with sacral intradural and epidural deposition. Type I familial amyloid polyneuropathy may cause amyloid deposition along the leptomeninges of the spinal cord and brain in addition to the visceral organs and the peripheral somatic and autonomic nerves.