The authors report histochemical and electron mciroscopic data of brain biopsies in two cases of Battern's disease. The siblings affected, a male and a female, are born from consanguineous North African parents. The diagnosis of Ceroïd-Lipofuscinosis is supported by neuro-glial, endothelial and perithelial autofluorescent cell storage. By electron microscopy the abnormal cytosomes show both curvilinear and finger-print profiles; their lysosomal nature is supported by their obvious acid phosphatase activity. These lipopigment cytoplasmic inclusions are compared with those described in senile brains and in other cases of Batten's disease.