Congenital glycolipidoses of the nervous system cause accumulation of various storage substances in cells. Methods of lipid and lectin histochemistry were used in an attempt for classification by known groups of diseases, on the basis of stored lipids. While conventional methods of lipid histochemistry enabled classification of accumulated substances only by lipid classes, lectin approaches provided additional information on sugar bonding points in cases of glycosphingolipidoses. For example, HPR-labelled and FITC-labelled WGA was positively recorded from neurons in cases of GM 2-gangliosidoses and mucopolysaccharidoses, type HURLER. Particular interest was aroused by RCA-I and PNA-positive reactions in glial cells of globoid cell leucodystrophy, since no histochemical method had been available in the past for diagnosis of this storage disease. Hence, the findings recorded by ALROY et al. (1986) from Twitcher mice, an animal model of galactosylceramidosis, have been confirmed by human material.