A procedure to extract and purify gangliosides from small volumes of plasma (0.6 mL), cerebrospinal fluid (1 mL) and fibroblasts is described. Gangliosides were extracted with chloroform/methanol and purified by means of reversed phase chromatography and gel filtration before analysis by thin layer chromatography. The procedure proved to be useful in confirming deficiency of lysosomal enzyme activity affecting ganglioside breakdown. The new procedure also appeared to be useful to monitor ganglioside catabolism in cultured fibroblasts loaded with ganglioside.