Human and canine globoid leukodystrophy are shown to be indistinguishable morphologically. Both have characteristic deposits with polygonal cross-sectional profiles in addition to twisted ribbon-like structures. The specificity of the deposits is emphasized, and their laminated nature is corroborated by the freeze-fracture studies. Their apparently hollow profiles in cross section are thought to be produced by the tight arrangement of the bilayers that prevent penetration of the stain.