The article reports on the pathology of GM2 gangliosidosis in Jacob sheep as the potentially new model of the Tay-Sachs disease (TSD) in humans. Information on this autosomal recessive lysosomal storage disease is provided along with TSD. The methods used in the study of four lambs are presented including the necropsy that followed. The results are explained highlighting severe histologic changes in the nervous system, markedly distended Purkinje cells with cytoplasmic material in the cerebellum, and neurons widely affected in the peripheral.